Beruflich Dokumente
Kultur Dokumente
SYSTEMIC DISEASES
By
Dr. Puneeta Vohra
CONTENT
SR.NO
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
TOPIC
Introduction
Gastrointestinal Disorders
Hematological Disorders
Endocrine Disorders
Immunological disorders
Nutritional Disorders
Renal Disorders
Infectious Disease
Sexually Transmitted Diseases
Bibliography
PAGE NO.
3-8
9-22
23-82
83-125
126-169
170-189
190-196
197-245
246-290
291-302
It is often said that mouth is the mirror of ones own health. The
manifestations, which occur in oral cavity for any systemic disease, are due
to its embryonic origin1. Dentistry is one of several areas of medicine
concerned with oral mucosal lesions which are many times indicator for
underlying systemic illness to make a suspected diagnosis. A number of
these may develop because of complication of or as partial manifestation of
underlying systemic disease. These may also occur due to patients
concurrent drug therapy for underlying systemic conditions.
It is now widely recognized that certain systemic disease such as diabetes
mellitus any many immune disorders may increase risk for periodontal
diseases. The hypothesis that oral conditions such as periodontal infections
may be risk factors or indicators for important medical outcomes represents
a paradigm shift in thinking about causality and the directionality of oral and
systemic association. This paradigm shift is encapsulated by new term the
periodontal medicine which refers to perspective that periodontal disease is
interrelated with systemic health in important ways.
The concept that oral infections such as periodontitis, caries and candidiasis
can adversely affect systemic health is not new. At the end of last century a
theory of focal infection developed, that local foci of infection were
responsible for initiation and progression of various inflammatory systemic
conditions like arthritis, sub acute bacterial endocarditis, peptic ulcers etc. a
significant recent advances in health care has been the movement towards a
evidence based practice. The important aspect of this is risk assement
involving classification of individual probability o acquiring disease.
The oral cavity might be thought to be the window to the body. Oral lesions
and symptoms are usually the result of local disease, but can be earliest
indication of, or in some instances may be the primary features in, patients
3
with systemic disease. Oral manifestations can some times lead to diagnosis
alternatively; systemic disease may require oral health care to be modified
for patients or operators safety. Oral cavity reflects the state of systemic
health more frequently than other parts of body. Even in ancient times
examination of mouth and tongue was given great importance. The oral
tissues are in direct physical continuity with rest of body and they are also
related via blood, lymphatics, and nerve pathway. Dentist has an important
role in preventive medicine as many systemic diseases have primary oral
manifestations.
Individuals in oral medicine participate in interdisciplinary medical care in
the areas of oral manifestations of systemic disease, oral oncology, pain,
histopathology which are frequently hospital-based services.
These oral manifestations must be properly recognized if the patient is to
receive appropriate diagnosis and referral for treatment. The lesions of the
oral mucosa, tongue, gingiva, dentition, periodontium, salivary glands, facial
skeleton, extra oral skin and other related structures are caused by some of
the more common systemic diseases.
Most of these manifestations are nonspecific but should alert the dentist to
the possibility of concurrent systemic disease or latent systemic disease that
may develop subsequently.
ORAL MEDICINE is the specialty of dentistry that is concerned with the
oral health care of medically compromised patients and with the diagnosis
and non surgical management of medically related disorders or conditions
affecting the oral and maxillofacial region. Oral medicine specialists are
concerned with the non surgical medical aspects of dentistry. These
4
of
medical
and
oral
health
care,
management
of
Last but not the least effectively communicate the importance of oral health
in the context of total health.
GASTROINTESTINAL DISORDERS
DISEASES OF THE UPPER DIGESTIVE TRACT
Gastro esophageal Reflux Disease
Hiatal Hernia
GASTROINTESTINAL SYNDROMES
Eating Disorders: Anorexia and Bulimia
Gardners Syndrome
Plummer-Vinson Syndrome
Peutz-Jeghers Syndrome
Cowdens Syndrome
INTRODUCTION
To provide safe and appropriate dental care, dentists are typically concerned
with the proper diagnosis of oral manifestations of gastrointestinal disorders,
homeostasis, risk of infection, drug actions and interactions, the patients
ability to withstand the stress and trauma of dental procedures, and proper
medical referral (when necessary).
The digestive system is composed of the esophagus, stomach, small
intestine, and large intestine. Each of these components performs specific
functions as ingested substances move through the different anatomic areas.
Additionally, the exocrine functions of the pancreas, liver, and gall bladder
combine to complete the assimilation of dietary calories and nutrients.
Both dentists and gastroenterologists have their primary focus within the
alimentary canal. The common embryogenesis of the oral cavity and
gastrointestinal tract is occasionally reinforced for the clinician when he or
she finds heterotopic gastric mucosal cysts in the oral mucous membranes or
on the tongue2,3. However, in addition to these relatively rare anomalies, the
9
12
hand, caries reveals soft, discolored dentin and results from the bacterial
breakdown of sugars on the surface of the teeth (Schroeder, 1995). The
prevalence of caries is not increased in persons with GERD, possibly
because the acidic environment interferes with the formation of the dental
biofilm. Good dental care and control of acid helps decrease the prevalence
of erosion. However, once the erosion occurs, it is irreversible and can only
be treated with surgical restorative procedures. Therefore, early recognition
and patient education is the most effective treatment.
Smoking cessation will increase the production of saliva and therefore
counteract the symptoms of GERD. Fatty meals slow down gastric emptying
and produce distention and reflux. An increase in the fat content of meals
may be an important factor in explaining Patients who have a diagnosis of
GERD may need to be treated in a semisupine position and premedicated
with H2 receptor antagonists or antacids.Any medications that may cause
nausea (such as narcotic analgesics) should be prescribed judiciously
because of the increased likelihood of regurgitation and possible
aspiration.Mild baking soda mouth rinses (one-half teaspoon of sodium
bicarbonate in 8 ounces of water) may be rinsed and expectorated to
minimize dysgeusia due to acid reflux. Topical fluoride applications via a
custom-made occlusive tray will ensure optimal dental minealization.
Treatment
Estrogen/progesterone combinations used in contraceptives and during
pregnancy also have been shown to decrease sphincter pressures. Proton
pump inhibitors (PPIs) such as omeprazole and (more recently) lansoprazole
have been found to heal erosive esophagitis more efficaciously than do H2
13
receptor antagonists. PPIs provide not only symptomatic relief but also
resolution of signs, including those that involve significant ulcers and/or
esophageal damage.8 Studies have shown that PPI therapy can provide
complete endoscopic mucosal healing of esophagitis at 6 to 8 weeks in 75 to
100% of cases. Daily PPI treatment provides the best long-term reduction of
symptoms for patients with moderate to severe esophagitis. Remission for as
long as 5 years has been seen. Promotility drugs are effective in the
treatment of mild to moderately symptomatic GERD. These drugs increase
loweresophageal sphincter pressure (which helps decrease acid reflux) and
improve the movement of food from the stomach.
15
16
17
GARDNERS SYNDROME
This genetic syndrome is chracterised by osteomas, fibromas, epidermoid
cysts includes polyposis. Gardners syndrome consists of intestinal polyposis
(which
represents
premalignant
lesions)
and
multiple
impacted
19
PEUTZ-JEGHERS SYNDROME
Peutz-Jeghers syndrome is characterized by multiple intestinal polyps
throughout the gastrointestinal tract but primarily in the small intestine.
Malignancies in the gastrointestinal tract and elsewhere in the body have
been reported in approximately 10% of patients with this syndrome.
Pigmentation (present from birth) of the face, lips, and oral cavity is a
hallmark of this syndrome.12 Interestingly, the facial pigmentation fades later
in life although the intraoral mucosal pigmentation persists. No specific oral
treatment is necessary.
COWDENS SYNDROME
Cowdens syndrome (multiple hamartoma and neoplasia syndrome) is an
autosomal dominant disease characterized chiefly by facial trichilemmomas,
gastrointestinal
polyps,
breast
and
thyroid
neoplasms,
and
oral
PYOSTOMATITIS VEGETANS
Oral lesions in mouth, related to inflammatory bowel disease, are termed
pyostomatitis vegetans, include deep fissures, pustules, ulcers, and papillary
projections. The course of these lesions tends to follow that of bowel
disease. Most patients with these lesions have ulcerative colitis or Crohns
disease. Some have liver disease Oral lesional biopsy and gastrointestinal
20
Pyostomatitis Vegetans
ulcerations on labial mucosa
COLOR PLATE-I
21
PEUTZ-JEGHERS SYNDROME
Pigmentation present on face and lip since birth
HAEMATOLOGICAL DISORDERS
22
LYMPHOMA
Hodgkins Disease
Non-Hodgkins Lymphoma
Burkitts Lymphoma
Oral and Dental Considerations
MULTIPLE MYELOMA
Treatment
Oral Manifestations
Dental Management
POLYCYTHEMIA
Polycythemia may be defined as an abnormal increase in the erythrocyte
count in the peripheral blood, usually accompanied by an increase in
hemoglobin and hematocrit. Polycythemia is divided into absolute
erythrocytosis (a true increase in red-cell mass) and relative erythrocytosis
(the red cell mass is normal, but the plasma volume is reduced). Relative
polycythemia is caused by the loss of tissue and intravascular fluid, which
may be the result of such diverse conditions as diabetic ketoacidosis,
postsurgical dehydration, prolonged vomiting or diarrhea, or rapid diuresis
secondary to treatment for congestive heart failure. In relative polycythemia,
the hemoglobin rarely rises more than 25gm%, and there are no appreciable
oral changes.
Three main groups of polycythemia are recognized: primary proliferative
polycythemia (polycythemia rubra vera), secondary polycythemia resulting
from changes in erythropoietin concentration, and apparent polycythemia.
The latter condition lacks a true increase in red-cell mass.
POLYCYTHEMIA VERA
Polycythemia vera (PV) is a myeloproliferative disorder characterized
by excessive proliferation of erythroid elements along with granulocytic and
megakaryocytic cells; it usually begins after 50 years of age. The etiology of
PV is unknown; however,it is likely a result of acquired genetic changes in
the stem cell leading to disturbances of normal cellular growth.
Oral Manifestations
A purplish red discoloration of the oral mucosa is visible on the tongue,
cheeks, and lips. The gingivae are red and may bleed spontaneously.
Petechiae and ecchymoses are observed in patients with platelet
abnormalities. Varicosities seen on the ventral surface of tongue, a frequent
24
ANEMIA
Introduction
Anemia is defined as an abnormal reduction in number of circulating red
blood cells, the quantity of hemoglobin and volume of packed red cells in
given unit of blood.20 This reduction in hemoglobin may result from blood
loss, as in common iron deficiency anemia, from increased destruction of red
blood cells, as in the hemolytic anemias, from decreased production of red
cells, as in pernicious and folic acid deficiency anemias, or from
combinations of these three.When there is a combination of causes, one
mechanism usually predominates. Anemias also may be classified according
to
their
pathophysiologic
macrocytic)of
the
red
basis:
cells
size
or
their
(microcytic,
normocytic,
hemoglobin
or
concentration
26
Thalassemia
Other hemoglobinopathies (eg, hemoglobin C and F)
V Erythrocyte defects associated with other disease
Folic acid and vitamin B12 deficiency anemias
ANEMIA OWING TO BLOOD LOSS
IRON DEFICIENCY ANEMIA
Iron is essential for synthesis of heme portion of haemoglobin. Iron
deficiency anaemia is caused by imbalance between iron intake and loss of
inadequate utilization.
Etiology
Inadequate intake of iron, malabsorption of iron, increased requirement of
iron in growing child and pregnancy, chronic blood loss such as menstrual
and menopausal bleeding.
Clinical features
Iron deficiency anemia (blood loss anemia, hypochromic microcytic anemia)
is the most common of all anemias, affecting approximately 30% of the
worlds population and accounting for up to 500 million cases worldwide. It
occurs chiefly in women in 4th and 5th decades of life. Nails become brittle
and flattened and often show spoon shape (koilonychias)17
Causes
28
Iron deficiency anemia may result from chronic blood loss, such as occurs in
menstrual or menopausal bleeding, parturition, bleeding hemorrhoids, or a
bleeding malignant lesion or ulcer in the gastrointestinal tract. It also may
develop in patients from a variety of causes that may decrease the rate of
absorption of iron, such as subtotal or complete gastrectomy, or a habit of
clay eating, or as part of malabsorption syndromes.18
Oral manifestations
The major oral sign of iron deficiency anemia is pallor of the mucosa due to
lack of oxygenated blood in capillary bed in lamina propria and is associated
with lower levels of hemoglobin. In addition, the oral epithelial cells become
atrophic, with loss of normal keratinization. The tongue may become smooth
due to atrophy of the filiform and fungiform papillae, and glossodynia can
be a presenting or associated symptom. In long-standing cases, esophageal
strictures or webs can develop, resulting in dysphagia. Recent clinical
investigation has shown lingual signs and symptoms to be much less
common than was previously believed of skin extending up to and beyond
mucocutaneous junction. Recurrent apthous ulceration and candidal lesions
can also occur in iron deficiency anaemia.
Histologic examination of the tongue mucosa shows a reduction in
epithelial thickness, with a reduction in the number of cells in spite of an
increase in the progenitor cell layer. The cell size is decreased in the
maturation layers (in males), and the nucleocytoplasmic ratio is higher than
normal. Lingual mucosal atrophy may occur in the absence of other clinical
findings.
COLOR PLATE-II
29
Diagnosis
30
Dental Considerations
Dental patients presenting with symptoms of anemia or oral signs suggestive
of this condition should have a complete blood count (CBC) with
differential. If significantly lowered hemoglobin values are obtained, the
patient should be referred to his or her physician for a more thorough
medical history, laboratory diagnosis, and treatment. Elective oral surgical or
periodontal procedures should not be performed on patients with marked
anemia because of the potential for increased bleeding and impaired wound
healing.When hemoglobin levels fall below 10 g/dL, the low oxygen tension
affects the rheologic interactions between the cellular components of blood,
mainly platelets and endothelium, decreasing their ability to clot effectively.
General anesthesia should not be administered unless the hemoglobin is at
least 10 g/dL. The patient should never be treated with iron until the cause of
the microcytic hypochromic anemia is found and corrected or until a
thorough search for the cause has proved fruitless.
Treatment
31
findings.
The
esophageal
lesions
are
demonstrable
32
infection,
particularly
pneumococcal
or
meningococcal,
and
abdominal pain, muscle and joint pan, at high temperature which may result
in circulalatory collapse. There is cardiomegaly, presence of leg ulcers and
gall stones. Most of persons expire before the age of 40. Sickle cell anaemia
should be suspected in black patients, particularly in those of afrocarribean
origin and investigated ,if anaesthesia is anticipated .if hemoglobin
concentration is less than 10gm/dl, then patient is probably a homozygote
with sickle cell disease, and hospitalization is necessary for anaesthesia .In
those with sickle cell trait the main precaution is that general anesthesia if
unavoidable should be carried out with full oxygenation .
Oral manifestations
Other than the jaundice and pallor of the oral mucosa, patients often show
delayed eruption and hypoplasia of the dentition secondary to their general
underdevelopment. Because of the chronic increased erythropoietic activity
and marrow hyperplasia, which are attempts to compensate for the
hemolysis, increased radiolucency resulting from the decreased number of
trabeculae is seen on dental radiographs. This change is noted especially in
the alveolar bone between the roots of the teeth, where the trabeculae may
appear as horizontal rows, creating a ladderlike effect . By contrast, the
lamina dura appears dense and distinct. In skull films, the diploe is
thickened, and the trabeculae are coarse and tend to run perpendicular to the
inner and outer tables, giving a radiographic appearance of hair on end.
The teeth do not present undue mobility.Areas of sclerosis or increased
radiopacity represent areas of past thromboses with subsequent bony
infarction. Patients with sickle cell anemia are particularly prone to develop
osteomyelitis, probably because of hypovascularity of the bone marrow
secondary to thromboses.
34
Diagnosis
FRESH BLOOD is sealed in a small chamber of microscopic slide with
metabisulfite (a reducing agent) for 1 hour and then observed for sickling.
Hemoglobin electrophoresis is less expensive, more accurate, and more
definitive in the diagnosis of sickle cell disease as it detects hemoglobin
S.10
Treatment
There is no treatment for sickle cell disease other than symptomatic
treatment. Regular health care and prompt antibiotic treatment of infections
are important. Painful bone infarcts should be treated with NSAID and fluid
intake should be increased . Patients with sickling disorders are more likely
to have pain which is indistinguishable from toothache presumably due to
pulpal infarcts. Therefore patients should be treated with analgesics in the
first instance unless an obvious carious lesion is present .Admission to
hospital is required for severe painful crises not responsive to analgesics .
THALASSAEMIAS(Cooleys anemia, Mediterranean anemia, and
Erythroblastic anemia)
The thalassemias are a group of congenital disorders characterized by a
deficient synthesis of either the or the chains of globin in the hemoglobin
molecule. As a result, the red blood cells are microcytic and hypochromic
with an aberrant morphology. Thalassemias are often considered among the
hypoproliferative anemias, the hemolytic anemias, and the anemias related
to abnormal hemoglobin. In -thalassemia (deficient or reduced chain)
intracellular inclusions, Heinz bodies are formed by the precipitation of the
chains that accumulate in excess following the impaired chain production. In
the most severe form of this disease, the fetuss red blood cells contain
35
36
37
Diagnosis
Hemolytic anemia with hypochromic microcytic red blood cells that vary in
size and shape is characteristic of thalassemia major. The hemoglobin
electrophoresis shows increased amounts of fetal hemoglobin and variable
amounts of normal adult hemoglobin. In patients homozygous for thalassemia, there is no detectable hemoglobin A. Prenatal diagnosis of
thalassemia is facilitated by deoxyribonucleic acid (DNA) analysis of
amniotic fluid cells, and it plays an important role in genetic counseling.
Treatment
Patients with mild thalassemia ( trait or minor) are clinically normal and
require no treatment. In other cases, the patients survival depends on blood
transfusions. Prevention of a hemoglobin concentration decrease to under 10
g/dL improves the chances of normal development and survival into
adulthood. This hypertransfusion treatment results in iron overload with
hemosiderosis and iron deposition in all body tissues.
Dental Management
As in any patient with a chronic anemia, poor healing may ensue after
surgical dental procedures. The possibility always exists of exacerbating the
symptoms of cerebral or cardiac hypoxia if substantial bleeding occurs in a
patient who is already anemic. Surgery has been used successfully to treat
the facial deformities.
ANEMIA OWING TO DECREASED PRODUCTION OF RED CELLS
MEGALOBLASTIC ANEMIA
The term megaloblastic anemia is used to describe a group of disorders
characterized by a distinct morphologic pattern in hemapoietic cells. These
cells have small immature nuclei and large mature cytoplasms.
38
39
Color plate-III
PERNICIOUS ANEMIA
Atrophy of papillae and lobulations on tongue
40
SIDEROPENIC ANEMIA
It is certain pathological condition in which iron gets accumulated in
mitochondria and appears as a ring of granules round the nucleus. These are
called as ring sideroblast and chracterised cell of sideropenic anemia which
is either hereditary or acquired.
APLASTIC ANEMIA
It is rare disorder chracterised by peripheral blood pancytopenia (anemia,
leucopenia, thrombocytopenia) associated with bone marrow suppression
Fanconis anemia is an inherited anemia that manifests in early childhood.
chracterised by pancytopenia, bone marrow hypoplasia, and congenital
anomalies. The management is to stop any drugs that may be responsible
and to give antibiotic and transfusions.
Oral Manifestations
Oral mucosa shows pallor. In some cases spontaneous hemorrhage may
occur from gingiva. Petechiae are often present on soft palate and in severe
cases submucosal ecchymosis. Large ragged ulcers covered by gray or black
necrotic membrane may be present, which are the result of generalized lack
of resistance to infection and trauma.
41
Hematological findings
RBC count is remarkably diminished as low as 1 million cells/mm3.
WBC and platelet count is as low as 2000/mm 3. Bleeding time is prolonged
clotting time is normal. Anemia is normocytic with some degeree of
macrocytosis.
Dental management
Gingival bleeding can be reduced by antithrombolytic agent such as
aminocaproic acid and transexemix acid 20mg/kg QID 24 hrs before the
procedure and continued for three days.
WHITE BLOOD CELL DISORDERS
The three types of granulocytes are neutrophils, eosinophils, and
basophils.Neutrophils are the most dominant of all circulating phagocytes
Lymphocytes are the primary cells involved in immunity. They appear to
originate from pluripotential stem cells in the bone marrow and migrate to
other lymphoid tissues, including lymph nodes, spleen, thymus, and mucosal
surfaces of the gastrointestinal tract. There are two types of lymphocytes
thymus-dependent
lymphocytes
and
non-thymus-dependent
42
LEUKEMIA
Leukemia, originally described by Virchow in 1874 as white blood, is a
malignancy affecting the WBCs of the bone marrow. This neoplastic process
is characterized by differentiation and proliferation of malignantly
transformed hematopoietic stem cells, leading to suppression of normal
cells. The malignant cells replace and turn off the normal marrow elements,
causing anemia, thrombocytopenia, and a deficiency of normally functioning
leukocytes. In time, the leukemic cells infiltrate other body organs,
destroying normal tissue.
Any of the white blood cells may be involved by this disorder and for this
reason disease is often classified as:
1.Lymphoid (lymphoblastic,lymphocytic) leukemia-involving the
lymphocytic series.
2.Myeloid(myelogenous)leukemia-involving progenitor cell that gives rise
to terminally differentiated cells of myeloid series (erythrocyte,granulocyte,
monocyte and platelets)
The classification may be modified to indicate the course of disease by
application of terms acute, sub-acute, and chronic.
Etiology
Virus-Epstein-Barr virus, herpes like virus and HTLV (human T cell
leukemic virus) have been considered to be the etiological agents
responsible for leukemia. Radiation and atomic energy over the dose of 100
rads, it is known to significantly increase the risk of leukemia. Leukemia
among radiologists and Japanese exposed to the atomic blast are more, as
compared to other population. Chronic exposure to aniline dyes, benzene
and phenylbutazone have been recognized to be associated with
43
may
result
from
thrombocytopenia
and
leukostasis
45
Acute Leukaemia,
Gingival Enlargement and Ulcerations
46
Hematological findings
The total WBC count may vary from a very low count less than 1 x 10 6per
cu mm to as high as 5000 x 10 6per cu mm or more. The peripheral smear
shows significant number of immature granulocytes or lymphatic precursors
or even stem cells. Bone marrow is hypercellular with replacement of
normal marrow elements by leukemic blast cells in varying degree.
There is an associated normochromic anemia, thrombocytopenia and
decrease in normally functioning neutrophils.
Management
First phase is phase of induction, pateint is treated using combination of
vincristine (1.4 mg/m2 every week of 1 month), L-asparaginase (600 units/m2
biweekly for 1 month) and prednisone (40 mg/m 2 orally daily for 1month).
As the leukemic cells regress, regrowth of normal cells occurs and the
patient goes rapidly into remission. Phase of consolidation in this, drugs
used include daunorubicin, mercaptopurine, cytarabine, and methotrexate
with intra-thecal therapy using the last two drugs, together with irradiation
of
the
cranium
to
eradicate
the
disease
from
central
nervous
CHRONIC LEUKEMIA
Chronic leukemias are characterized by the presence of large leukemic cells
and differentiated WBCs in the bone marrow, peripheral blood and other
tissues. It has a prolonged clinical course even without therapy.
48
49
50
Oral manifestations
The most common oral finding is hypertrophy of gingiva. There may be
ulceration with necrosis and gangrenous degeneration, a dark brown exudate
and foul fetor oris are present. Tongue is frequently swollen and dark.
Regional lymphadenopathy is seen. Rapid loosening of teeth due to necrosis
of periodontal ligament has been reported. Destruction of alveolar bone also
occurs in some cases.
Hematological findings
Peripheral blood smear shows mild anemia and a large number of small
lymphocytes. Lymphoblasts are rare but increase in the terminal stages of
disease. WBC count may increase up to 1000 x 106 per cu mm.
Management
General measure to maintain good health, adequate rest, good food and
exercise should be advised. In chemotherapy, chlorambucil 6 to 10 mg/day
for 14 days with break of 14 days and cyclophosphamide 2 to 3 mg/kg IV
for 6 days. Combination therapy of cyclophosphamide, doxorubicin,
vincristine, and prednisone have been recommended.Radiotherapy with very
small doses, of only 150 rads over a period of five weeks, is very effective
and may induce satisfactory remission. Steroids are given if the bone
marrow is severely involved initial treatment with prednisone 40 mg daily
and 25-50 mg daily later should be given.
Radiographic features of leukemia
It affects the entire body as it is malignancy of bone marrow. It is presented
as ill defined patchy radiolucent area. There is destruction of alveolar,bone
loss may be in form of transverse lines of increased radiolucency or irregular
areas and loss produces the so called, moth eaten appearance. Sclerosis of
bone may be presented alone or in combination with destructive lesion. In
51
52
ALEUKEMIC LEUKEMIA
It is the sub-leukemic form of leukemia in which the WBC count of the
peripheral blood is normal or even subnormal and abnormal or immature
leukocytes may be present.
Dental Considerations
Topical treatment to stop gingival bleeding like removal of local irritants,
direct pressure and use of absorbable gelatin, collagen sponge, topical
thrombin and placement of microfibrillar collagen. If these local measures
are not successful platelet transfusion is given. Platelet transfusion and
intravenous combination of antibiotics is required before any dental
treatment.
Oral Ulcers
Oral mucosal ulcers are common findings in leukemic patients taking
chemotherapy and are frequently caused by the direct effect of
chemotherapeutic drugs on the oral mucosal cells. The ulcers are
characteristically large, irregular, and foul smelling, and are surrounded by
pale mucosa caused by anemia and a lack of normal inflammatory
response. The most common cause of oral ulcers in leukemic patients
receiving chemotherapy is recurrent HSV infections. These infections
involve the intraoral mucosa and the lips. Lesions frequently begin with the
classic cluster of vesicles typical of recurrent HSV and quickly spread,
causing large ulcer. The management of non-HSV oral ulcers in leukemic
patients should prevent the spread of localized infection, minimize
bacteremia, promote healing, and reduce pain. The ulcers in hospitalized
leukemic patients taking chemotherapy may be infected with organisms not
commonly associated with oral infection, particularly gram-negative enteric
bacilli. Topical antibacterial treatment can be attempted with povidone53
iodine
solutions,
bacitracin-neomycin
ointments,
or
chlorhexidine
54
PRIMARY AGRANULOCYOSIS
The etiology of primary agranulocytosis is unknown. Secondary
agranulocytosis is used when cause is recognized. Mild neutropenia when
1000/mm3 to 2000/mm3 neutrophils are present. Moderate neutropenia when
500/mm3 to 1000/mm3 neutrophils are present. Idiosyncrasy or sensitization
to certain drugs like aminophylline, chlorpromazine and phenylbutazone,
benzene, bismuth, chlorampheenicol, sulfonamides and use of cytotoxic
drugs or antimetabolics, deficiency of vitamin B12 and folic acid. Certain
infections decrease the number of neutrophils in circulating blood because of
increased migration of neutrophils into the tissue, destruction of neutrophils
or direct effect of microorganism and its toxins on the bone marrow.
Infection with hepatitis A and varicella zoster virus infection are commonly
associated with neutropenia. Overwhelming bacterial infection, particularly
septicemia can be accomplished by neutropenia because cells are used at
rapid rate to overcome infection. Diseases causing sequestration of
neutrophils includes systemic lupus erythematosus and Feity's syndrome. It
can be associated with leukemia, pancytopenia and hypersplenism.
Hemodialysis patient experience decrease in neutrophil owing destruction by
complement activated by the dialysis membrane. Irradiation can cause
neutropenia due to direct toxic effect on division of bone marrow cells.
Clinical features
It can occur at any age but it somewhat more common in adults, particularly
in woman. It is also common in professional and in hospital as they have
easy access of the offending drugs and often use drug sample injudiciously.
Symptoms may be sudden or gradual. The condition begins with sore throat,
55
high fever and often rigors, which may be followed by prostration. Skin
appears pale and anemic and in some cases, jaundiced. There is rapidly
advancing necrotic ulceration of throat and mouth with little evidence of pus
formation In most of the cases patient dies within 3 to 5 days due to toxemia
and septicemia.
Oral manifestations
The most common sites are gingiva and palate, tonsil and pharynx.
There may be associated pain, excessive salivation and spontaneous oral
hemorrhage. The oral lesions appear as necrotizing ulcerations of oral
mucosa, tonsils and pharynx. The lesions appear as ragged and necrotic and
are covered with a gray black membrane. The necrotic tissue is often foul
smelling. On margins of lesion there is lack of inflammation.The disease
spreads quickly in gingival tissues causing destruction of supporting
structures and inevitable loss of deciduous and permanent teeth.
Supporting alveolar bone is rapidly destroyed, so that teeth are denuded of
bone and are supported only by soft tissues. Very rarely, infection spreads to
deep into the marrow to cause osteomyelitis.
Hematological findings
Majority of patients show a leukocyte count below 2000 cells per cu mm 3
and granulocyte count below 100 cells per cu mm3. Hemoglobin and platelet
counts are normal.
56
CYCLIC NEUTROPENIA
In cyclic neutropenia there is fall in the number of circulating neutrophils at
regular intervals of 3-4 weeks. It is therefore necessary to monitor the white
cell count daily for several weeks inorder to diagnose the condition. It may
cause oral ulceration and periodontal breakdown. Etiology is drugs and
chemicals infections and long term exposure to radiation. In Oral cavity
spontaneous hemorrhage may occur from gingiva. Petechiae may be seen in
soft palate.
Treatment
The universally accepted treatment for most cases of cyclic neutropenia is
careful monitoring of the patient for infection during neutropenic periods
and vigorous early management of infection. In some patients, use of
corticosteroids, adrenocorticotropin, or testosterone modulates the sharp
reduction in marrow function.Unfortunately, these drugs are not successful
for all patients. The use of granulocyte colony-stimulating factor (G-CSF)
has been employed to boost neutrophil levels. Unlike with congenital
agranulocytosis, G-CSF therapy in cyclic neutropenia is not associated with
the development of acute myeloid leukemia or myelodyplasia.24
Oral and Dental Considerations
Oral lesions are common in cyclic neutropenia and may be the major
clinical manifestation of the disease. The two most common oral
manifestations are oral mucosal ulcers and periodontal disease. The oral
ulcers recur with each new bout of neutropenia and resemble the large deep
scarring ulcers seen in major aphthous stomatitis. The periodontal
57
by
syndrome
is
rare
oculocutaneous
autosomal
albinism,
recessive
progressive
defect
neurologic
58
ORAL MANIFESTATIONS
The gingivae appear intensely red, despite the neutropenia, with
granulomatous margins. Severe gingival recession is common, and early
severe periodontal disease with advanced bone loss, mobility, denuded roots,
and loss of teeth. These patients may also report recurring oral ulcerations
that may correspond to neutrophilic count.
LYMPHORETICULAR MALIGNANCY
Primary malignant changes in lymphoreticular tissue is of two main types:
Hodgkins disease accounts for about 20% of cases and non hodgkins
lymphoma for about 80%. The presentation is usually enlargement of the
lymph nodes with a rubbery consistency. Oropharyngeal involvement is rare
in hodgkins disease less than 1%of cases but nonhodgkins lymphoma more
commonly presents in the mouth and can be oral manifestation of HIV
disease
Types
1.Hodgkin's lymphoma
2.Non-Hodgkin's lymphoma
HODGKINS LYMPHOMA
It was first described by British pathologist Thomas Hodgkin in 1832. It is
characterized by painless enlargement of lymphoid tissue throughout the
body.
Etiology
Particularly herpes and oncorna virus are being investigated as possible
etiological agents. Sometimes, it can occur without any etiological factor.
59
Clinical features
It is characterized by a bimodal age incidence, peak one in young adults and
the second in the 5th decade of life with equal distribution between sexes.
The onset is insidious, usually with enlargement of one group of superficial
nodes. The cervical lymph nodes are usually the first to be involved but the
disease may start in the mediastinal, axillary, abdominal, pelvic or inguinal
lymph nodes. The involved nodes are painless. Generalized weakness, loss
of weight, cough, dyspnea and anorexia are seen.Pain in back and abdomen
owing to splenic enlargement, due to pressure of enlarged nodes or
involvement of vertebrae. The lymph nodes are discrete and rubbery in
consistency with overlying skin being freely mobile. Splenomegaly is
usually seen in later stage. Some patients may manifest pruritis.
Characteristic features of this disease are Pel-Ebstein fever, a cyclic spiking
of high fever and generalized severe pruritis of unknown etiology. Pressure
of enlarged lymph nodes on adjacent structures may cause dyspnea,
dysphagia, venous obstruction, jaundice and paraplegia.
Clinical stages (ANN Arbor staging)
Stage I- involvement of single lymph node region or extra-lymphatic sites.
Stage II- involvement of two or more lymph node regions or an extra
lymphatic site and lymph node region on the same side of diaphragm.
Stage III- involvement of lymph node region on the both sides or without
extralymphatic involvement or involvement of spleen or both.
Stage IV-diffuse involvement of one of more extralymphatic tissues e.g.
liver or bone marrow.
Such stages are subdivided into A and B categories depending on whether
they have systemic symptoms such as weight loss, fever, night sweats.
60
Oral manifestations
Primary jaw lesions are uncommon. Secondary effect can be seen in oral
cavity in the form of infection due to reduced host immune response.It may
appear in the oral cavity as an ulcer or a swelling or as an intra-bony lesion
which presents as a hard swelling.
Histopathological features
It is characterized by replacement of normal lymph node architecture by an
admixture of malignant lymphoid cells and non-neoplastic inflammatory
cells. Reed-Sternberg cells which are sheets of lymphoid cells with
interposed
vacuolated
spaces
containing
characteristic
bi-nucleated
seen in the vertebrae and pelvis. In it, there is frank sclerosis with filling of
the marrow spaces by bone. It presents as greyness or whiteness which is
abnormal. The margins may be well defined and sharp or irregular and
trailing off gradually into the normal bone.
Laboratory investigations
Anemia which is normocytic and normochromic is a common finding. The
total WBC count is normal but there may be mild eosinophilia. In the
terminal stage there, may be leukopenia and thrombocytopenia. ESR is
raised. Liver function may be abnormal due to infiltration in liver. LDH is
raised level is an adverse prognostic factor. Chest radiography is done to
permit staging.
Management
Radiotherapy irradiation treatment 3500-4000 rads/week over, the involved
region plus all adjacent sites been given in stage I and II. It is also given
after chemotherapy, to sites where there was originally bulk disease.
Chemotherapy is given in stage III and IV. Usually combination is given.
First combination is MOP, i.e. mustine HCl (6 mg/m 2 IV on day 1 and day
8), oncovin which is also called as vincristine (1.4 mg/m 2 IV on day 1 and
8), procarbazine (100 mg/m2) orally from day 1 to 14) and prednisone (40
mg/m2 orally from day 1 to 14). MOPP combination given in six courses
with no drugs is given from day 15 to 28. Second combination is ABVD
regimen, i.e. adriamycin (25 mg/m 2 IV bolus on day 1,8 and 14), bleomycin
(10 mg/m2 bolus on day 1,14) vinblastine (6 mg/m 2 IV bolus on day 1,14)
and decarbazine (375 mg/m2 IV bolus on day 1,14). The cycle should be
repeated on 20th day. A combination of radiotherapy and chemotherapy may
increase the overall response and long-term survival but, it is associated with
62
Non-Hodgkin's Lymphoma
It is also called as lymphosarcoma. In this group, there is neoplastic
proliferation of lymphoid cells, usually affecting the B-lymphocytes. Unlike
Hodgkin's lymphoma, the disease is frequently widespread at the time of
diagnosis, often involving not only the lymph nodes but also bone marrow,
spleen and other tissue. Early involvement of bone marrow is typical of this
lymphoma.
Types
1. Nodular
2. Diffuse
Etiology
The etiology is unclear but herpes viral etiology has been suggested. There
may be induced immunologic effect permitting a malignant clone to
proliferate.
Clinical Features
It affects persons of all ages from infants to the elderly.But is commonest in
middle age group. Males are affected more commonly than the females.
In the oral cavity it frequently occurs in tonsils. The other sites affected are
salivary glands or jaws. The onset of symptoms may be insidious.
Painless lymph node enlargement of abdominal and mediastinal region is the
most common finding. Very often the first group of lymph nodes affected
may be cervical, axillary or inguinal. The patient complains of tiredness, loss
63
64
COLOR PLATE-V
65
Histopathological features
In the nodular pattern the neoplastic cells tend to aggregate in such a way
that large clusters of cells are seen. Diffuse pattern is characterized by a
monotonous distribution of cells with no evidence of nodularity or germinal
center pattern.
Laboratory investigations
Blood count usually shows normal levels but if there is associated
hypersplenism or hemolytic anemia the reduced WBC and RBC counts are
seen along with reduced hemoglobin levels and reticulocytosis. In some
cases there may be slight increase in lymphocytes and thrombocytopenia.
Moderate degree of anemia will also present when there is considerable bone
marrow involvement. Some very aggressive high grade non-Hodgkin
lymphomas are associated with very high urate levels which can precipitate
renal failure.
Management
No treatment is necessary, if disease is not advanced. When diagnosed in late
stages, chemotherapy is the treatment of choice. In most cases single agent
chemotherapy
(chlorambucil)
is
usually
given.
Combination
with
66
67
68
PLATELET DISORDERS
PLATELET DISORDERS ARE CLASSIFIED AS
A.PURPURA
Idiopathic thrombocytopenic purpura
Secondary thrombocytopenic purpura
Congenital purpura
Thrombocytopathic purpura
Von willebrands disease
Bernard-soulier syndrome
Aldrich syndrome
B. Thrombocytosis
PURPURA
It is defined as purplish discoloration of the skin and mucous
membrane due to subcutaneous and submucus extravasation of blood.
Purpura may be due to defective platelets or due to an unexplained
increase in capillary fragility.
IDIOPATHIC THROMBOCYTOPENIC PURPURA
69
Treatment
70
Clinical features
71
Oral manifestations
72
Gingival bleeding and postextraction bleeding are the most common oral
manifestations. The disease may be discovered after dental extraction.
Hematological findings
Prolonged bleeding time, normal platelet count, failure of aggregation of
platelets impaired adherence of platelets and depressed levels of factor VlII
are suggestive of this disorder. Low AHG levels and abnormal platelet
retention to glass beads. The platelet count and prothrombin consumption
are normal. The clotting time usually is normal but capillary fragility is
increased.
Management
Bleeding episode is best controlled by transfusion of plasma and or
cryoprecipitate and by local control of homeostasis. Patients with von
Willebrand's disease should not be given aspirin because of prolongation of
the bleeding time that may occur.
BERNARD-SOULIER SYNDROME
It is transmitted as an autosomal dominant trait and is characterized by
prolonged bleeding time and defective aggregation of platelet. The only
treatment is platelet transfusion during acute bleeding episodes.
ALDRICH SYNDROME
It is also called as Wiskott-Aldrich syndrome. It is X linked recessive
condition.
Clinical features
73
Laboratory finding
Bleeding time is prolonged while clot retraction is impaired. Platelet count
is normal as the clotting time. The aggregation of platelet by epinephrine,
ADP and thrombin is defective.
Management
No specific treatment but patient in oral surgery can be given microfibriallar
collagen preparation with fibrinolytic inhibitors.
THROMBOCYTOPATHIC PURPURA
It occurs due to qualitative defect in platelets.
Clinical features
They have severe bleeding tendency and bruise easily after minor trauma.
Spontaneous ecchymosis is common. Epistaxis and bleeding into
gastrointestinal tract are common findings.
Oral manifestations
Spontaneous gingival hemorrhage with mucosal ecchymosis occasionally
occur. Excessive and prolonged bleeding form dental extractions may pose
serious management problems.
Laboratory findings
Platelet count is normal but bleeding time is either normal or prolonged,
which is due to defective platelet aggregation, so that normal capillary
plugging is impaired.
Management
75
THROMBOCYTOSIS OR THROMBOCYTHEMIA
It is characterized by an increase in the number of platelets in the blood in
excess of 1000 x 106/dl. It is of two types, i.e. primary and secondary.
Causes
Polycythemia and myeloid leukemia, anemia, tuberculosis and sarcoidosis.
Hyperadrenalism, rheumatoid arthritis and bronchial carcinoma.
Clinical features
It is a rare disorder of the elderly associated with a tendency to bleed and to
have thrombic episodes. Epistaxis, bleeding into the gastrointestinal tract as
well as bleeding into the genitourinary tract and central nervous system is
common. Hemorrhages in skin are also common.
Oral manifestations
Spontaneous gingival bleeding and excessive and prolonged bleeding after
extraction of tooth is also common.
Laboratory findings
The platelet count is increased and there is abnormal aggregation in response
to several aggregating agents.
76
Color plate-VI
THROMBOCYTOPENIA,
purple colored petechiae and echhymosis
77
THROMBASTHENIA
HEMORRHAGIC DISORDERS
HEMOPHILIA A
It is a hereditary disorder of blood coagulation characterized by excessive
hemorrhage due to a prolonged coagulation time. Deficiency of factor VIII
or antihemophilic factor is the cause of hemophilia. It is transmitted as Xlinked recessive character carried on X-chromosome. The males are
clinically affected and the females are carriers of the trait. Hemophilia A
occurs 10 times more commonly than hemophilia B.
Types
Mild-less than 4 percent of AHG
Moderate-1 to 3 percent of AHG
Moderate to severe-O.O% to 0.9% of AHG
Severe-O% of AHG
Clinical features
Bleeding manifestations usually begin after 6 months of age, when the child
begins to move about and tends to fall and sustain injuries, i.e. when
spontaneous hemorrhage is noted by the parents.The most common
manifestation is hemorrhage into joints which is spontaneous and associated
with warmth and muscle spasm. Repeated episodes cause damage to the
joint with wasting of the related muscle, leading to deformity and crippling.
Hemorrhage into subcutaneous tissues, internal organs and musculature also
are frequent and potentially disabling complications. Superficial trauma
78
Oral manifestations
Traumatic injury of the oral cavity may lead to the diagnosis of hemophilia.
The anatomic sites involved in persistent oral bleeding are the frenum of lip
and the tongue. There is prolonged bleeding after tooth extraction.
Hematoma of the floor of mouth may occur and blood may spread via the
fascial spaces and produce a hematoma of the larynx, with consequent
respiratory embarrassment. Physiological processes of tooth eruption and
exfoliation may be associated with severe and prolonged hemorrhage.
Gingival hemorrhage is extremely rare and when it does occur, it is the result
of gingival injury.
Hematological findings
Clotting time is prolonged, but however the bleeding time, platelet count and
prothrombin time are all normal.The prothrombin consumption time and the
partial thromboplastin time may be prolonged in severe cases.
Management
The main aim is to raise the factor VIII levels which can arrest bleeding.
Replacement therapy-various forms of replacement therapy are available
like plasma cryoprecipitate and factor VIII concentrates. Hypovolemia,
allergic reaction, transfusion hepatitis, hemolytic anemia and development
of factor VIII antibodies are complications with fresh frozen plasma.
Factor VIII concentrates-the dose and duration of administration of factor
VIII concentrates are planned, taking into consideration the site and the type
of bleeding.Intraligamentary injection in hemophilia does not cause
79
81
Oral manifestations
Spontaneous gingival bleeding occurs in some cases and prolonged bleeding
after extraction of tooth is observed.
Laboratory findings
Both clotting and prothrombin time are prolonged but, bleeding time is
normal. The basic defect is reduction in plasma proaccelerin.
Management
Transfusion and freshly frozen plasma are given when there is excessive
hemorrhage.
Anticoagulant Treatment
Coumarin anticoagulants such as warfarin are used for prevention e.g. of
thromboembolic disease, which can complicate atrial fibrillation or insertion
of prosthetic heart valves. The underlying condition may therefore influence
oral health care management more then anticoagulant treatment. The latter
should be checked regularly to maintain prothrombin time, which is reported
as international normalized ratio of 2-3 in most cases but 3-4.5 for those
with prosthetic valves. Short term anticoagulation with heparin is given
before renal dialysis session. Heparin is effective only for about 6hrs.
Extraction or other surgery can therefore be delayed for 12-24hr after the
last dose of heparin, when the benefits of dialysis are also maximal.
82
ENDOCRINE DISORDERS
HYPOTHALAMUS AND ANTERIOR PITUITARY
Introduction and Pathophysiology
DISEASES OF PITUTARY GLAND
HYPERPITUITARISUM
Gigantisum
Acromegaly
HYPOPITUTARISUM
THYROID DISEASE
General Description
Pathophysiology
Hyperthyroidisum
Hypothyroidisum
Management
Prognosis
Oral Health Considerations
PARATHYROID DISEASE
Hyperparathyroidisum
Hypoparathyroidisum
DISEASE OF PANCREATIC GLAND
Diabetese mellitus
Type-I(IDDM)
Type-II(NIDDM)
ADRENAL GLAND DISORDER
Addisons Disease
Cushings Syndrome
Adrenal Insufficiency
83
84
GIGANTISUM
Clinical Features
Generalized overgrowth of most tissue in childhood. Most of the soft tissue
and bones respond to the excess hormone by enlarging. Excessive
generalized skeletal growth. Patient may often have of height to 7 to 8 feet.
Patients achieve monstrous size because of tumors of the pituitary gland.
Later in life it may show genital underdevelopment and excessive
perspiration and they complain of headache, lassitude, fatigue, muscle and
joints pain and hot flashes. There is increase in size of calvarium which may
85
lead to change in the hat size. Pituitary tumors may also induce deficiency of
other pituitary hormones causing signs of hypogonadism including
decreased libido and menstrual problems in women.
ACROMEGALY
It is more common in males and occurs most frequently in 3rd decade.
Bone overgrowth and thickening of the soft tissue cause a characteristic
coarsening of facial features termed acromegaly. Hand and feet become
large, with clubbing of the toes and fingers due to enlargement of the tufts of
the terminal phalanges. There is temporal headache, photoophobia and
reduction in vision. The terminal phalanges of the hands and feets become
large and the ribs also increase in size.
Oral Manifestations
Teeth
Teeth in gigantism are proportional to the sized of jaw and the rest of the
body and root may be longer than normal. The teeth become spaced, partly
because of enlargement of the tongue and party because upper teeth are
situated on the inner aspect of the lower dental arch, due to disproportionate
enlargement of the two jaws
Jaw bone
Mandibular condylar growth is very prominent. Overgrowth of mandible
leading to prognathism. Mandible may be of extraordinary proportions
creating a major discrepancy between the upper and lower jaws and a
pronounced class III malocclusion. In some cases the growth at the condyle
exceeds that of the alveolar processes, so that increased in vertical depth of
the ramus is greater than that of the body of the jaw, consequently the upper
86
and lower teeth fail to come into proper occlusion.The palatal vault is
usually flattened and the tongue increase in size and may cause crenation on
its lateral border. The lips become thick and Negroid. In edentulous patients
enlargement of the alveolus may prevent the comfortable fit of complete
dentures.
Radiographic Features
Skull changes
Enlargement of sella turcica, enlargement of paranasal sinus and excessive
pneumatization of temporal bone squames and petrous ridge. Diffuse
thickening of outer table of skull. Enlargement and distortion of the pituitary
fossa. Air sinus-the air sinuses are really prominent in acromegaly rather
than in gigantism.
Teeth
Increased tooth size especially root due to secondary cemental hyperplasia.
Diastema between teeth due to lengthening of dental arch. Increase in
thickness and height of alveolar process.
Jaw bone
In acromegaly the angle between the ramus and body of mandible may
increase, which results in anterior tooth root push forward so they appear as
fan out. There is also lengthening of condylar process, class III skeletal
relationship. The new bone laid down on the condyle results in an increase
in the vertical length of the ramus as well as overall length of whole bone.
It is greater on the tip and posterior aspect of the coronoid processes, on the
posterior border of the ramus and on the chin. Enlargement of the mandible
is common finding as the length of the horizontal and ascending rami both
87
88
Diagnosis
89
Complete absence of third molar bud. Roots of teeth are short and apices are
wide open and pulp canal is toward the apex. There is loss of alveolar bone.
Management
It is usually directed towards removal of the cause or replacement of the
pituitary hormone or those of its target glands.
92
features are specific, but there is an inverse relationship between the age at
which treatment is started and the degree of mental retardation.
The signs and symptoms of hyperthyroidism are the result of increased
secretion of T4 by the thyroid gland, but many are identical to the signs and
symptoms of anxiety. In the dental patient in pain, signs of hyperthyroidism
may coexist with and exacerbate the patients normal response to pain and
anxiety. In addition, routine examination of the head and neck may disclose
signs of thyroid disease, including changes in oculomotor function,
protrusion of the eyes, excess sweating, enlargement of the thyroid or the
tongue, lingual thyroid tissue, and difficulty in swallowing. Treatment of
thyroid disease can accelerate the protrusion of the eyes and can cause
agranulocytosis.29Atrial fibrillation, increasing thyroid size, and swings in
thyroid hormone levels to symptomatic hypothyroid or hyperthyroid status
are also possible during medical therapy for underlying thyroid disease.
Pathophysiology
The thyroid is a unique endocrine gland that depends on dietary iodine
intake to produce the hormone thyroxine. Thyroxine regulates the pace of
metabolism in all cells through interactions with mitochondrial, nuclear and
extramitochondrial processes.37 Dietary iodine intake modulates the
functional activity of the thyroid gland, directly altering thyroid sensitivity
to TSH as well as producing an inhibitory effect on thyroid function
independent of TSH.38 Iodine deficiency results in increases in thyroid gland
size (goiter) and hypothyroidism. The major circulating form of thyroid
hormone is T4, but T3, the tri-iodinated variation of thyroxine, is the active
intracellular form of the hormone.37 The location of the thyroid gland (at the
base of the neck, just superior to the sternal notch) permits easy observation
93
94
HYPERTHYROIDISM
It is also called as thyrotoxicosis and it is a syndrome in which there is
excessive production of thyroxin in thyroid gland. It is associated with
diffuse toxic goiter and less frequently with toxic nodular goiter or toxic
adenoma. Excessive thyroxin causes generalized increase in metabolic rate
of all body tissues. In patient with thyrotoxicosis, dental treatment can
precipitate an acute emergency like thyroid crisis or thyroid storm.
Definition
Hyperthyroidism is excessive functional activity of the thyroid gland that
can be caused by Graves' disease, excessive replacement of or overdose of
thyroid hormone.
Causes
Exophthalmic goiter-It is characteristic by diffuse hyperplasia of the thyroid
and by eye signs.
Toxic adenoma-It is the result of hyperfunction which
originates by a
95
Clinical Features
It has predilection for females between 20 and 40 years of age. Thyroid is
diffusely enlarged, smooth, possible asymmetrical and nodular, a thrill may
be present, may be tender. Abdomen, liver and spleen may be enlarged.
Neuromuscular-It includes nervousness, fine tremors, and muscle weakness,
mood swings from depression to extreme euphoria, emotional liability,
hyper-reflaxia, ill sustained clonus, proximal myopathy, bulbar myopathy
and periodic paralysis.
Gastrointestinal
Weight loss despite normal or increased appetite, diarrhea, bowel alterations,
anorexia, vomiting and hyperdefecation.
Cardiorespiratory
Palpitation, excessive perspiiration, irregular heart beat. Increased metabolic
activity leads to increased circulatory demands, tachycardia and increased
pulse pressure and sometimes congestive cardiac failure. Exertional dyspnea
and ankle edema, blood pressure normal, systolic hypertension may be
present. Angina and cardiomyopathy and exacerbation of asthma.
Ocular
In thyrotoxicosis patient may have bulging eye and partial paralysis of the
ocular muscles, retraction and jerky movement, corneal ulceration, optic
neuritis, ocular muscle weakness, papillooedema, loss of visual activity,
exophthalmos.
Reproductive
Amenorrhea, oligomenorrhea, infertility, spontaneous abortion and loss of
libido, impotence.
96
Dermatological
Increases sweating, pruritus, oncholysis, pigmentation, vitiligo, digital
clubbing and pretibial myxedema (bilateral nonpiiting edema).
Other features include heat intolerance, sweaty and warm extreemities, thin
shiny skin, pretibial myxedema, increased PR and early fatigue,
lymphadenopathy, thirst and osteoporosis.
Oral Manifestations
Advanced rate of dental development and early eruption with premature loss
of primary teeth. Generalized decrease in bone density or loss of some areas
of edentulous alveolar bone. Early jaw development and alveolar bone
atrophy.
Radiographic Features
In older children and adults well marked generalized osteoporosis is
sometime appears but it is not revealed in the jaw. In some cases there may
be alveolar resorption and in some cases there may be greater density of the
trabeculae. .
Laboratory Investigation
Plasma levels of T3 and T4 are increased, free thyroxin index is raised in this
disorder. Thyroid stimulating hormone (TSH) is decreased. Anemia may be
moderate to severe degree and is seen in patient with prolonged duration of
the disease. The anemia is hypochromic and abnormal forms of RBC may be
seen.
Management
Antithyroid drugs-It would be appropriated to give antithyroid drugs for 12
to 18 months to those in whom a single episode was anticipated.
Carbimazole-For 0 to 3 weeks-40 to 60 mg daily in divided doses, for 4 to 8
97
antagonist-selective
receptor
antagonist
such
as
HYPOTHYROIDISUM
It is caused by insufficient secretion of thyroxin by the thyroid gland. As
failure of thyrotrophic function on the part of the pituitary gland or an
atrophy or destruction of the thyroid gland leads to an inability of the thyroid
to produce sufficient hormone to meet the requirement of the body.
Definition
Decreased or deficient secretion of thyroid hormones caused by, thyroiditis,
insufficient thyroid replacement, postthyroidectomy, postradioactive iodine
therapy.
Types
Cretinism-If failure of hormone occurs in infancy.
Juvenile myxedema-If it occurs in childhood.
Myxedema-If it occurs after the puberty. In it there is subcutaneous
deposition of hydrophilic mucoopolysaccharides.
98
99
Oral Manifestations
Cretinism and juvenile myxedema
Teeth
Dental development delayed and primary teeth slow to exfoliate. Enamel
hypoplasia can also be seen. Abnormalities of dentin formation lead to
enlarge pulp chamber.
Jaw bone
Maxilla is overdeveloped and mandible is underdeveloped. Retarded
condylar growth leads to characteristic micrognathia and open bite
relationship.
Tongue
It is enlarged by edema fluid and may protrude continuously and such
protrusion may lead to malocclusion of teeth.
The base of skull is shortened leading to a retraction of the bridge of the
nose with flaring. Face is wide and fails to develop in longitudinal direction.
Lips are puffy, thickened and protruding.
Myxedema
Tongue and lip-Macroglossia and enlarged lip are seen as a result of the
deposition of water and protein including facial swelling of non pitting type
and mandible is underdeveloped. There is greater tendency to periodontal
disease, with alveolar destruction and loosening of the teeth.
Radiographic Features
Skull bones show delayed closing of the frontanels and epiphysis, numerous
wormian bones. There is transverse line of increased density involving the
metaphysical regions. Teeth reveal thinning of lamina dura. Delayed dental
eruption, short tooth root. Separation of teeth as a result of enlargement of
tongue, periodontal diseases, loss of teeth and external root resorption. The
100
101
COLOR PLATE-8
102
105
develops
this
condition
known
as
tertiary
Radiographic Features
Demineralization of skeleton bone matrix contains less than normal amounts
of calcium producing unusually radiolucent skeletal image. There is lack of
normal contrast in the radiograph resulting in over all grayness, often
associated with a granular appearance in the bone. The rarefaction is of
homogeneous nature and there may be normal, granular or ground glass
appearance.Sometimes rarefaction gives a mottled or moth eaten appearance
with varying density. Osteitis fibrosa generalisata localized destruction of
bone is produced by osteoclastic activity leaving residual area of fibrosis. In
some cases moth-eaten appearance can be seen.
Brown tumor
It may develop peripherally or centrally, it appears radiographically as ill
defined radioolucency called as brown tumor, as gross specimen is brown or
reddish brown. In it, trabeculae are completely missing. It may occur in
pelvis, ribs or femur but are most commonly found in facial bones and jaws.
These lesions may be multiple within the single bone or they may be
polyostotic. They appear as unilocular or multilocular with variably defined
margin and may produce cortical expansion.
Pathological calcification-Punctuate and nodular calcifications occasionally
occur in kidneys and joints. Earliest change is subtle erosion of bone from
subperiosteal surface of phalanges of hand.
Skull bones-Entire calvarium has granular appearance caused by loss of
central trabeculae and thinning of cortical tables. Evidence in the skull vault
of osteopenia producing a fine overall stippled pattern to the bone, hence it
107
serum
alkaline
phosphatase
level
is
elevated
in
primary
108
HYPOPARATHYROIDISM AND
PSEUDOHYPOPARATHYROIDISM
It is an uncommon condition in which there is insufficient secretion of
parathyroid hormone. Pseudohypoparathyroidism is a condition in which
there is defect in the response of tissue target cell to normal level of
parathyroid hormone.
Etiology
Surgical damage to parathyroid gland and their vascular supply during
thyroid gland procedure. Parathyroid damage from radioactive iodine 131.
Autoimmune destruction of parathyroid gland.
Clinical Features
Hypocalcemia-it can lead to tetany in the form of carpopedal spasm of the
wrist and ankle joint including stiffness in hands, feet and lips. There is also
paresthesia of hand, feet and around the mouth. Tingling in the circumoral
area, fingers ad toes. Patients may complain of anxiety, depression, epilepsy
and chorea. Reduction in intellectual capacity due to calcification within the
brain.
Pseudohypoparathyroidism
Patient with pseudohypoparathyroidism manifests short stature due to early
closure of certain bony epiphysis. The face is round and the hand shows
shortening of the metacarpal bones, so that fingers are short.
Trousseau's sign
109
110
COLOR PLATE-9
111
112
Polyphagia-Excessive hunger.
Breathe-Presence of acetone breathe.
Visual activity-Visual difficulty ranging from proogressive color blindness
to total blindness that have disease more than 20 years.
Atherosclerosis-Coronary
artery
disease
and
stroke
are
frequent
complication.
Diabetic neuropathy-It can cause marked irritability.
Infection-Recurrent vaginal (yeast) infections, recurrrent urinary tract
infections, recurrent skin infections (especially of feet) and reversible
paresthesia of fingers or toe.
Other symptoms inclue nocturia, weight loss, fatigue, obesity usually present
in older age group, nausea; vomiting. Temperature, blood pressure may be
elevated and peripheral pulses may be reduced.
Oral Manifestations
Gingival and periodontal disease
It will influence the onset and course of periodontal disease. Patient with
diabetes are more prone to develop periodontal disease than are those with
normal glucose metabolism. The patient may exhibit a fulminating
perioodontitis with periodontal abscess formation and inflamed painful
abscess and even hemorrhagic gingival papillae, this factor culminated and
give rise to tooth mobility, i.e. loose teeth. It will show more severe and
rapid alveolar bone resorption and are more prone to develop periodontal
abscess. Insulin dependent diabetic children tend to have more destruction
around the first molars and incisors than elsewhere. As such diabetes
mellitus does not cause periodontal disease directly but it alters the response
of the periodontal lesion to local irritants, hastening bone loss and retarding
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116
altered taste sensation, oral lichen planus, and diffuse enlargement of parotid
gland.
COLOR PLATE-10
LESIONS IN DIABETES MELITUS
117
Radiographic Features
118
119
Fat intake should be reduced; the use of monounsaturated oils in the diet
(e.g. olive oil, peanut oil) is beneficial.
Reduced intake of sodium (no more than 6 g/ day), alcohol abstinence.Low
calories and sugar free drink are useful for patients with diabetes.
Oral hypoglycemic drugs-if dietary management proves ineffective in
controlling hyperglycemia. hypoglycemic drugs like insulin or oral
hypoglycemic are prescribed.
Sulfonylurea
First generation sulfonylurea in which tolbutamide is nowadays is given in a
dosage of 25 or 500 mg 8 or 12 hourly. It is useful in the elderly in whom the
risk and the consequence of inducing hypoglycemia are increased. In second
generation sulfonylurea, gliclazide and glizipide are widely used.
Biguanides
They are less widely used than sulfonylurea because of their higher
incidence of side effects. Mechanism of action is by increasing insulin
sensitivity and peripheral blood glucose uptake. Metformin in dose of 500
mg 12 hourly. Its use is contraindicated in persons with impaired renal or
hepatic function and in those who take alcohol in excess because of risk of
lactic acidosis.
Alfaglucoside inhibitors
They delay carbohyydratesabsorption in the gut by selectively inhibiting
disaccharides. Acrabose is currently available in a dose of 50 to 100 mg with
each meal.
Insulin therapy
120
122
123
Oral Manifestation
The pale brown or deep chocolate pigmentation of the oral mucosa,
spreading over the buccal mucosa from the angle of the mouth and/or
developing on the gingiva, tongue, lips may be first evidence of disease.
Laboratory Investigation
The associated anemia is normocytic and normochromic associated with
reticulocytosis. There is reduction in the red cell mass. There is high blood
levels potassium and low concentration of sodium and chloride. Elevated
blood urea nitrogen.
Management
Glucocorticoids replacement-Cortisol is the drug of choice. In patients who
are not critically ill hydrocortisone 15 mg on waking and 5 mg at 6 PM in
evening. Supplement mineralocorticoid can be given.
CUSHING'S SYNDROME
Cushing's syndrome arises from excess secretion of glucocorticoids by the
adrenal glands. It was described by Harvey Cushing in 1932.
Etiology
Cushings syndrome is mainly seen in cases of adrenal adenoma, adrenal
carcinoma, adrenal hyperplasia and basophilic adenoma of the anterior lobe
of pituitary gland. Pateint under medication of exogenous corticosteroid.
In cases of ACTH secreting tumor of the anterior pituitary associated with
adrenal cortical hyperplasia, in ectopically located adrenal like tumor like in
ovary and in alcoholics, major depressive illness and primary obesity.
Clinical Features
124
COLOR PLATE-11
125
Drugs
126
127
IMMUNOLOGIOCAL DISORDERS
Immunologic Disease
PRIMARY IMMUNODEFICIENCIES
Oral Manifestations
Dental Management
CONNECTIVE-TISSUE DISEASES
Systemic Lupus Erythematosus
Scleroderma
Dermatomyositis
Rheumatoid Arthritis
Miscellenous
Angiodema
Orofacial Granulomatosis
128
Many of the immunologically mediated diseases have some oral health care
relevance. Immunological disease will be considered under the headings of
allergy, atopic disease and autoimmune disease. The immune response, so
necessary for protection against disease, can also cause disease or other
undesirable consequences when it reacts against tissues.
IMMUNODEFFICIENCY DEFECT WITH PRIMARY DEFECT
IN CELLULAR IMMUNITY
DIGEORGE SYNDROME (VELOCARDIOFACIAL SYNDROME)
DiGeorge syndrome has recently been shown to be one of a group of
disorders caused by a deletion on chromosome 22q11. 55This genetic defect
results in abnormal development of the facial and neural crest tissues,
causing abnormal development of derivatives of the third and fourth
pharyngeal pouches. The result of this defect in embryonic growth are
abnormalities of the thymus, the parathyroid glands, and the great vessels of
the heart. The subsequent malfunction of these organs accounts for the
respective features of DiGeorge syndrome
Clinical features
Variable
immunodeficiency,
neonatal
hypocalcemia
secondary
to
DNA repair mechanisms common to these patients may account for the high
incidence of malignancies. The ATMgene, therefore, has the potential for
wide-ranging roles such as detecting DNA damage, preventing genomic rearrangements in malignancy, and preventing programmed cell death. The
immunologic abnormalities of AT include T-cell and B-cell deficiencies,
causing both an abnormal cellular response and a deficiency of
immunoglobulins. Due to a markedly hypoplastic thymus, the peripheral Tcell pool is frequently reduced in size. Although the number and class
distribution of B lymphocytes are usually normal, about 70% of AT patients
are serum IgA deficient and can also be deficient in IgG2 and IgG4. These
immunologic abnormalities may be the result of cells that exhibit
chromosomal breaks (usually in chromosomes 7 and 14) at the sites of the Tcell receptor genes and the genes that encode the heavy chains of
immunoglobulins. Treatment for AT is limited to supportive care as no cure
is available. Unless a severe IgG deficiency is present, therapy with gamma
globulin is not indicated.Due to the highly variable incidence of infection,
bone marrow transplantation is usually not advised.
WISKOTT-ALDRICH SYNDROME
Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by
lymphocytes and platelets that are faulty due to an altered cell surface
glycoprotein they share. The classic clinical features include a microcytic
thrombocytopenia, severe eczema, and pyogenic and opportunistic
infections. The immunologic findings of WAS are a result of both T-cell
defects and abnormal immunoglobulin levels. The T cells have a uniquely
abnormal appearance due to a cytoskeletal defect. Moreover, the T cells are
defective in function, and this malfunction becomes progressively worse.
132
135
CONNECTIVE-TISSUE DISEASES
Connective-tissue diseases are customarily grouped together under names
such as collagen disease, collagen vascular disease, hyperimmune disease, or
autoimmune
disease.
rheumatoid
arthritis,
They
include
scleroderma
systemic
lupus
(progressive
erythematosus,
systemic
sclerosis),
these
diseases.
In
connective-tissue
diseases,
the
term
but
some
investigators
136
believe
that
viruses
or
other
Clinical Manifestations
SLE is a disease with multiorgan involvement. Immunocomplex deposition
causes small-vessel vasculitis, which then leads to renal, cardiac,
hematologic, mucocutaneous, and central nervous system destruction. In
addition, inflammation of the serous membranes results in joint, peritoneal,
and pleuropericardial symptoms. As there is no typical pattern of
presentation, one patient may present with dermatitis and kidney disease
whereas another may present with arthritis, anemia, and pleurisy. Thus,
whenever a patient demonstrates signs and symptoms of multiorgan
involvement, SLE should be considered in the differential diagnosis,
especially for a female who is 20 to 40 years of age. The following is a brief
overview of the most frequently encountered clinical manifestations.
Renal Manifestations
Kidney involvement in the form of glomerular destruction is seen in
approximately 50% of patients. The glomerulonephritis results from the
deposition of complement and immunocomplexes in the basement
membrane of the glomerulus. Five to twenty-two percent of SLE patients
progress to end-stage renal disease and require hemodialysis or
transplantation.77 Nephrotic syndrome results from massive destruction and
is a common cause of death in SLE patients. The severity of kidney disease
is often a good indication of the overall prognosis of the patient.
Cardiac Manifestations
Accelerated atherosclerosis and valvular heart disease constitute the primary
cardiac manifestations of SLE. The most common of all cardiac lesions in
SLE patients involves the endocardium and was originally described (by
Libman and Sacks) as verrucous valvular lesions. Lupus-related valvular
pathoses can include valve leaflet thickening,with or without regurgitation.
140
Hematologic Manifestations
The primary hematologic diseases among SLE patients are leukopenia,
anemia, and thrombocytopenia. Leukopenia (< 4,000/mm3) is common and
usually reflects lymphopenia but can also be due to immunosuppressive
therapies. Anemia of chronic disease occurs in most patients during periods
of disease activity but is also often due to hemodialysis.Hemolytic anemia
occurs in a small proportion of patients with positive Coombs test results.
Thrombocytopenia (< 100,000/mm3) results from increased phagocytosis of
autoantibody-coated platelets by spleen, liver, bone marrow and lymph node
macrophages and can occur in up to 25% of patients.When antiphospholipid
antibodies or the lupus anticoagulant and anticardiolipin antibodies are
present, patients are prone to episodic thrombosis, thrombocytopenia, and
spontaneous abortion.
Mucocutaneous Manifestations
The cutaneous manifestations of SLE include photosensitive rashes,
alopecia, Raynauds phenomenon, and skin ulceration secondary to
vasculitis. Cutaneous involvement does not necessarily correlate with
increased systemic disease. The malar or butterfly rash (which affects
fewer than half of SLE patients) and the discoid rash are the two most
characteristic rashes of SLE. The malar rash is a fixed flat or raised
erythematous rash over the cheeks and bridge of the nose, often involving
the chin and ears. It is usually exacerbated by ultraviolet light. A more
diffuse maculopapular rash, mainly in sun-exposed areas, is also common.
Vasculitic skin lesions include subcutaneous nodules, ulcers, and infarcts of
skin or digits. Oral mucosal lesions can be found as annular leukoplakic
areas and/or erythematous erosions or chronic ulcerations, often resembling
lichen planus.
141
Musculoskeletal Manifestations
Arthritis and arthropathies are the primary musculoskeletal disorders
associated with SLE. Arthralgia with morning stiffness is the most common
initial manifestation of SLE.More than 75 % of SLE patients develop a true
arthritis, which is symmetrical and non-erosive and which usually involves
the hands,wrists, and knees. Deforming arthritis is uncommon in SLE
patients.
Central Nervous System Manifestations
Significant neuropsychiatric signs and symptoms are found in 10% to 20%
of patients who have SLE.78 Diffuse and focal cerebral dysfunctions
(including psychoses, seizures, and cerebrovascular accidents) in addition to
peripheral sensorimotor neuropathies, account for more than 60% of
neuropsychiatric manifestations.79 Central nervous system involvement is a
poor prognostic sign.
Diagnosis And Laboratory Evaluation
The most important diagnostic laboratory test for SLE is the test for
antinuclear antibody (ANA) in the serum, which is positive for 96% to 100%
of patients with SLE. The clinician should remember that the ANA test is
also positive in a minority of patients with scleroderma or rheumatoid
arthritis, therefore, the diagnosis must be made on the total clinical picture,
not on a single laboratory test. In addition, SLE is characterized by the
production of numerous autoantibodies, including ANAs, antinative DNA,
rheumatoid factor, antibody to Smith (Sm) antigen, antibody to Ro (SS-A)
antigen, and antibody to La (SS-B) antigen. Many of these autoantibodies
produce specific laboratory and clinical abnormalities and can also be seen
in a variety of other rheumatologic diseases. An individual with elevated
142
ANA and antinative DNA most likely has lupus. Important findings on
routine laboratory tests include anemia, thrombocytopenia, (which may
occasionally be severe enough to cause purpura), increased levels of
globulins, and a biologic false-positive result on the serologic test for
syphilis (STS). Depressed complement levels (particularly C3 and C4),are
also common.
Oral Manifestations
Patients with SLE are affected by a variety of orofacial disorders, including
characteristic oral lesions, nonspecific ulcerations, salivary gland disease,
and temporomandibular disorders. The reported incidence of these
manifestations varies considerably, depending on the criteria of the
investigators. The first report of oral manifestations of SLE in the American
dental literature was in 1931, by the dermatologist Monash, who reported a
50% incidence of oral lesions.45 More recently, Rhodus and Johnson
reported a higher incidence (81.3% to 87.5%) of various oral lesions,
including ulceration, angular cheilosis,mucositis, and glossitis. They also
reported a high incidence (75.0% to 87.5%) of signs and symptoms of oral
conditions, including glossodynia, dysgeusia, dysphagia, and dry mouth.46
The oral lesions of SLE are caused by vasculitis and appear as frank
ulceration or mucosal inflammation. Some individuals with SLE or discoid
lupus have discoid-appearing oral lesions.82 The lip lesions often have a
central atrophic and occasionally ulcerated area with small white dots,
surrounded by a keratinized border composed of small radiating white striae.
The intraoral lesions are somewhat different because of the thinner
epithelium; they are composed of a central depressed red atrophic area
surrounded by a 2 to 4 mm elevated keratotic zone that dissolves into small
white lines. The oral lesions of SLE are easily confused with the lesions of
143
144
Treatment
With regard to the management of the oral lesions of SLE, there have been
no reports involving the treatment of a large series of patients. In general, the
oral ulcerations of SLE are transient, occurring with acute lupus flares.
Symptomatic lesions can be treated with high-potency topical corticosteroids
or intralesional steroid injections. Discoid lupus lesions on the lower lip. A
chronic palatal lesion (the initial sign of systemic lupus erythematosus in
this patient). Chronic lesion of the dorsal tongue in a patient with systemic
lupus.
Dental Consideration
Because SLE can be a widespread disease affecting many organ systems, the
dental management of an SLE patient requires a good understanding of
general medicine. The more common problems seen in SLE patients are
discussed below.
Adrenal Suppression
Patients with SLE may be taking adrenal-suppressive doses of
corticosteroids, which makes these patients susceptible to shock.
Glucocorticosteroid therapy will cause adrenal suppression that affects
adrenal function for up to 12 months, but the patients stress response will
return within 14 to 30 days. There is no need for replacement therapy for
dental treatment in patients who have not taken glucocorticosteroids during
the preceding 30 days. Patients who are receiving alternate-day therapy can
be treated on an off day without supplementation if they have been on the
alternate-day regimen for at least 2 weeks. Patients who are receiving daily
low-dose corticosteroid therapy (< 30 mg hydrocortisone equivalent) will
not need replacement therapy. Patients who are receiving daily high-dose
corticosteroid therapy (> 30 mg hydrocortisone equivalent) should be treated
145
and
prothrombin
time/partial
thromboplastin
time
Renal Disease
Renal disease is common in SLE patients and ranges from an asymptomatic
state to frank renal failure, therefore, the dentist should be aware of the
patients renal function (ie, creatinine clearance, serum creatinine, and blood
urea nitrogen). Patients who are undergoing hemodialysis should receive
dental treatment on nondialysis days.86
Exacerbation by Surgery
The dentist should proceed with caution in performing elective surgery or
dental procedures, especially in patients who have a history of postsurgery
lupus flares.
Exacerbation by Drug Therapy
Drugs that have been related to acute lupus flares include penicillin,
sulfonamides, and nonsteroidal anti-inflammatory drugs (NSAIDs) with
photosensitizing potential. All of these should be used judiciously.
147
SCLERODERMA
It is also called as Systemic Sclerosis or Hidebound disease. It is a
multisystem connective-tissue disease that involves hardening of the skin
and mucosa, smooth-muscle atrophy, and fibrosis of internal organs. The
prevalence of scleroderma is estimated to be about 250 per million,with
women being affected significantly more frequently than men.87 Several US
studies have suggested that black patients have a higher age-specific
incidence rate and more severe disease than have white patients.88
Nomenclature (Subtypes)
Localized scleroderma refers to scleroderma primarily involving the skin,
with minimal (if any) systemic features. Only rarely have patients with
localized scleroderma developed systemic sclerosis. Three major types of
localized scleroderma exist: morphea, generalized morphea, and linear
scleroderma. Morphea begins with violaceous skin patches that enlarge,
become indurated, and eventually lose hair and the ability to sweat. Later in
the course of the disease, the lesion burns out and appears as a hypo- or
hyperpigmented area depressed below the level of the skin.89 A small number
of patients develop numerous larger lesions that coalesce, and these
patients are said to have generalized morphea. Patients with either type of
morphea usually have a benign course characterized by the softening of the
lesions over time. Linear scleroderma is a form of the localized disease that
may develop during childhood and that usually involves the arms, legs, or
head. This form of the disease develops as a thin band of sclerosis that may
run the entire length of an extremity, involving underlying muscle, bones,
and joints.When the disease crosses a joint, limitation of motion is possible,
148
149
more than a nuisance, but some patients have recurrent episodes that are
associated with digital pitting scars, nail fold infarcts, or digital ulcers.
Cutaneous Manifestation
The thickening of the skin of PSS patients always begins in the fingers.
Early skin changes, starting with pitting edema, often involve the whole
hand and the extremities. In several months, the edema is replaced by a
tightening and hardening of the skin, which results in difficulty in moving
the affected parts.Hyperpigmentation, telangiectases, and subcutaneous
calcifications may also occur, leading to deformity and severe cosmetic
problems.
Musculoskeletal Manifestations
Polyarthralgias and morning stiffness affecting both small and large joints
are frequent in patients with scleroderma. Inflammatory joint pain with
markedly swollen fingers often appears to be true synovitis and can lead to
the premature diagnosis of rheumatoid arthritis.
Gastrointestinal Manifestations
Distal esophageal motor dysfunction is the most frequent gastrointestinal
finding; it results from weakness and incoordination of esophageal smooth
muscle and leads to distal dysphagia. Intestinal fibrosis leading to severe
intestinal malabsorption can also occur.
Cardiac Manifestations
Clinical evidence of myocardial involvement in scleroderma cases is
uncommon, but such involvement is more frequent in patients with diffuse
scleroderma. The clinical presentations of cardiac involvement can include
pericarditis, conduction problems, and congestive
Pulmonary Manifestations
Pulmonary interstitial fibrosis is now the most frequent cause of death in
patients with scleroderma since renal disease has become a treatable
complication. Patients with either limited or diffuse scleroderma can develop
interstitial disease although it tends to be more severe in patients with
diffuse scleroderma. In patients with severe fibrosis, the greatest damage
occurs during the first 5 years of illness, often when there are no pulmonary
symptoms. Pleural thickening, pleural effusions, and pneumothorax are less
frequent manifestations of lung disease in patients with scleroderma.58
Renal Manifestation
Until recently, renal involvement was the most dreaded and deadly
complication of scleroderma. The use of high-dose corticosteroids for the
treatment of scleroderma has been implicated in precipitating renal crisis in
some patients. In addition, pathologic changes due to the disease itself
typically show alterations resembling hypertensivenephrosclerosis as well as
mucinoid hyperplasia and vascular fibrinoid necrosis in the interlobar
arteries.Renal crisis is characterized by malignant hypertension, which can
rapidly progress to renal failure. The use of angiotensin-converting enzyme
inhibitors has helped to make renal disease due to scleroderma a very
treatable condition.
152
Laboratory Evaluation
ANAs are present in approximately 90% of scleroderma patients and are
characteristically antinucleolar or anticentromere antibodies. There are a few
other important ANAs that are specific for scleroderma but are not yet
commercially available. Anti-ribonucleic acid (RNA) polymerase III may be
the most common antibody found in patients with scleroderma. Other
laboratory findings include anemia, an elevated erythrocyte sedimentation
rate, and hypergammaglobulinemia.
Treatment
The treatment of PSS depends on the extent and severity of skin and organ
involvement. D-penicillamine, a drug effective for both rheumatoid arthritis
and Wilsons disease, has shown promise in the management of PSS by
decreasing both skin thickening and organ involvement. This drug has two
mechanisms of action: interference with cross-linking of collagen and
immunosuppression. Nifedipine is a calcium channel blocker that has been
shown to be effective in managing Raynauds phenomenon and myocardial
perfusion.94 Extracorporeal photochemotherapy has also shown promise in
reversing cutaneous sclerosis in patients in the early stages of PSS.95
Oral Findings
The clinical signs of scleroderma of the mouth and jaws are consistent with
findings elsewhere in the body. The lips become rigid, and the oral aperture
narrows considerably.96 Skin folds are lost around the mouth, giving a mask
like appearance to the face. The tongue can also become hard and rigid,
making speaking and swallowing difficult. Involvement of the esophagus
causes dysphagia.97 Oral telangiectasia is equally prevalent in both limited
and diffuse forms of PSS and is most commonly observed on the hard palate
and the lips.98 When the soft tissues around the temporomandibular joint are
153
154
Dental Management
The most common problem in the dental treatment of scleroderma patients is
the physical limitation caused by the narrowing of the oral aperture and
rigidity of the tongue. Procedures such as molar endodontics, prosthetics,
and restorative procedures in the posterior portions of the mouth become
difficult, and the dental treatment plan may sometimes need to be altered
because of the physical problem of access. The oral opening may be
increased an average of 5 mm by stretching exercises. One particularly
effective technique is the use of an increasing number of tongue blades
between the posterior teeth to stretch the facial tissues. In addition,
mechanical devices that assist the patient in performing the stretching
exercises are available. If these approaches are insufficient, a bilateral
commissurotomy may be necessary. When treating a patient with diffuse
scleroderma, the extent of the heart, lung, or kidney involvement should be
considered, and appropriate alterations should be made before, during, and
after treatment. Patients with extensive resorption of the angle of the
mandible are at risk for developing pathologic fractures from minor trauma,
including dental extractions. Patients with Sjgrens syndrome should have
daily fluoride treatments and make frequent visits to the oral hygienist.
155
DERMATOMYOSITIS
Dermatomyositis (DM) is a rare inflammatory degenerative disease
characterized by skin lesions and progressive muscle atrophy. The disease
occurs most frequently during childhood and between the fourth and sixth
decades of life. The true incidence and prevalence are difficult to ascertain
because of the rarity of the disease and the lack of consistent diagnostic
criteria. Most studies have found a preponderance of female patients over
male patients. Skin manifestations have been identified in 30% to 40% of
adult patients and in 95% of affected children.97 DM is classified with the
connective-tissue diseases because of many overlapping clinical features and
the fact that it is frequently seen together with scleroderma, SLE, rheumatoid
arthritis, or Sjgrens syndrome.
Nomenclature (Subtypes)
The three types of idiopathic inflammatory myopathies are DM,
polymyositis (PM), and inclusion body myositis. Specific subtypes of DM or
PM can be categorized as adult idiopathic, juvenile, or amyopathic DM.
There is also a form of DM that is associated with connective-tissue disease
or malignancy.
Etiology AND Pathogenesis
The etiology of DM is unknown, but genetic, immunologic, and
environmental factors are likely to play an important role. Studies of
histocompatibility antigen prevalence have demonstrated that human
leukocyte antigens HLA-B8, HLA-B14, and HLA-DR3 are associated with
dermatomyositis, but these studies have failed to link HLA haplotype with
disease.67Immune mechanisms play a significant role in the onset of the
disease, particularly circulating immunocomplexes, cellular immunity, and
autoantibodies to skeletal-muscle myoglobin or myosin. The onset of the
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158
Treatment
An underlying carcinoma should be ruled out in all cases of DM since it is
present in 10 to 25% of patients. Initial therapy consists of bed rest
combined with high doses of systemic corticosteroids. In resistant cases,
plasmapheresis or immunosuppressive drugs such as methotrexate or
azathioprine have been helpful.98
Dental Management
The disease process of DM poses no significant challenge to the dentist. The
same precautions as are necessary for all patients who are taking high-dose
long-term steroids and antimetabolites should be taken.
159
RHEUMATOID ARTHRITIS
Rheumatoid Arthritis (RA) is a disease characterized by inflammation of the
synovial membrane. Women are approximately three times more likely to be
affected than men, and 80% of people with RA develop signs and symptoms
of the disease at between 35 and 50 years of age. Epidemiologic studies
suggest that the incidence of the disease is declining in younger age groups
because of unknown environmental factors.99 Unlike degenerative joint
disease (osteoarthritis), which is localized to the joints in middle-aged and
elderly individuals, rheumatoid arthritis affects people of all age groups and
affects other organs, including muscles and the hematopoietic system.
Although this disease is called arthritis, there are frequent extra-articular
manifestations.
Nomenclature (Subtypes)
Several variations of RA exist. Feltys syndrome accounts for less than 5%
of the total cases. In addition to having the usual manifestations of RA,
patients with Feltys syndrome also have splenomegaly and leukopenia, with
neutrophils showing the greatest decrease. In severe cases, recurrent
bacterial infection is a common cause of death. Another variant is juvenile
RA (Stills disease), which is thought by some rheumatologists to be a
separate disease and not just a simple variation of adult RA. 70 Systemic
extra-articular symptoms are prominent, including fever, lymphadenopathy,
hepatosplenomegaly, carditis, and rash.Visual impairment secondary to
iridocyclitis (inflammation of the iris and ciliary body) may also occur. In
50% of patients, growth and sexual maturation are delayed during active
stages of the disease.
160
161
Infectious Factors
Several infectious agents (including both bacteria such as streptococci and
Mycoplasma, as well as viruses such as Epstein-Barr virus) have been
suggested to cause the initial T-cell influx.
Clinical Manifestations
The initial symptom in a majority of patients with RA is nonspecific
weakness and fatigue, which may precede joint symptoms by several
months. This is followed by symmetric polyarthritis characterized by a
complaint of stiffness and a finding of a spindle-shaped swelling of the
involved joints. The proximal interphalangeal joints of the fingers and
metacarpophalangeal joints of the hands are most commonly involved the
wrists, elbows, knees, and ankles also are frequently affected. In some
patients, all joints may be involved, including the temporomandibular joint
and the cricoarytenoid joint of the larynx. The joints that are affected with
RA become red, swollen, and warm to the touch.Muscle atrophy around the
affected
joint
is
common.
Extracapsular
manifestations
include
162
Oral Manifestations
The treatment of RA can cause oral manifestations. The longterm use of
methotrexate and other antirheumatic agents such as D-penicillamine and
NSAIDs can cause stomatitis. Cyclosporine may cause gingival overgrowth.
Direct effects of the disease are also seen. Patients with long-standing active
RA have an increased incidence of periodontal disease, including loss of
alveolar bone and teeth. Similarities in the host immune responses of RA
and periodontal disease, involving reduced cellular and enhanced humoral
activity, have been reported102 although the increased dental and periodontal
disease may be chiefly related to a decreased ability to maintain proper oral
hygiene. Sjgrens syndrome is a common complication of RA.
JUVENILE RHEUMATOID ARTHRITIS
It is also called as Still's disease and juvenile polyarthritis. It is defined as a
chronic synovitis with or without extraaarticular manifestations, but it is
accompanied by more systemic features than for adults.
Clinical Features
Age and sex-it occurs in children and has its peak between 1 and 3 years.
Joint involved-initial bilateral polyarthritis of both small and large joints
including the cervical spine. Signs-splenomegaly, lymphadenopathy,
leukoocytosis, pyrexia and rash.
Symptoms
Neck pain and a limited range of movement.
Restricted opening of the mouth.
TMJ involvement-one or both TMJ are involved.
Micrognathia-it may cause interference with normal condylar growth,
leading to micrognathia. This. is seen in at least 20 percent of cases.
163
progression
in
164
rheumatoid
arthritis
in
Investigations
Diagnosis and Laboratory Evaluation
Rose-Waaler test is positive in 70 percent of the patients with rheumatoid
arthritis.
Antinuclear
immunofluorescence.
antibodies
are
detected
by
indirect
166
ANGIODEMA
It is also called as Angioneuratic edema, Quinckes edema, Giant cell
urticaria) it is a diffuse edematous swelling of soft tissue commonly
involving the subcutaneous and submucosal connective tissue.Angiodema
may be hereditary, acquired or idiopathic in nature.
Heridetery angiodema: It is a rare autosomal dominnat genetic disorder
due to a C1 esterase inhibtor defect this leads to an unimpeded complement
response in inflammation, causing oedema due to stress , allergy or trauma.
Acquired angiodema: It can rise due to abnormal antibodies against C1
esterase inhibtor, or more commonly as a reaction to drugs such as
angiotensin-converting enzyme inhibitors, non-steroidal anti-inflammatory
drugs, food additives or latex rubber. Because of potential seriousness of
condition, patients require urgent management with airway maintenance to
treat the acute attack. Patients should then be refferd to a specialist. Those
with heridetery angiodema can be treated prophylactically with stanzol or
fresh frozen plasma (containing C1 esterase inhibtor), and those with
acquired angiodema require avoidance advice and the availability of self
administered steroids, adrenaline or antihistamines.
Clinical features
Clinically, there is a recurrent non-itchy oedema commonly affecting the
face,tongue,pharynx and larynx. This can lead to respiratory embarrassment,
and in severe cases, death. Angiodema manifests as a soft, nontender, diffuse
edematous swelling of relatively rapid onst, which may be solitary or
multiple, most commonly involving the face around the lips,tongue, pharynx
167
and larynx. Eyes may be swollen shut and lips extremely puffy. The skin
may be normal color or slightly pink. A feeling of tenseness or an itching or
prickly sensation sometimes precedes the swelling. In heridetery form
patients become symptomatic at second decade of life and this form is more
dangerous due to involvement of respiratory and gastrointestinal symptoms.
Treatment
When the etiological factor such as food can be discovered its elimination
from diet will prevent recurrent attacks. Antihistaminic drugs should be
administered. If the attack is not controlled or if laryngeal involvement is
present intramuscular epinephrine is given.
168
COLOR PLATE-12
Periocular angiodema
169
OROFACIAL GRANULOMATOSIS
Orofacial granulomatosis also can be called as orofacial lymphoedema.Signs
and symptoms are lip swelling, facial swelling, angular chelitis, mucosal
tags cobblestoning ulceration, apthae, full thickness gingivitis and palatal
hyperplasia. The lip and facial swelling is permanent but may increase or
decrease dramatically in response to exposure to allergens. When the lip
swelling worsens, tissues become more turgid due to oedema in the tissues,
and the lip swelling becomes exaggerated. There is almost always an
associated bilateral angular chelitis which improves with steroid therapy.
The intraoral buccal cobblestoning and mucosal tagging is also the result of
intence lymphoedema. The ulceration may be typically apthous in nature,
especially if there is concururrent nutrition deficiency (which is common, for
example, in intestinal crohns disease, or there may be deep intransigent
ulceration which persists indefinitely without healing. The full thickness
gingivitis and less commonly hyperplastic palatal inflammation, is due to
granulomatous inflammation, and is particularly severe in the mixed
dentition phase. Soft tissue swellings occasionally arise due to areas of
nonspecific infection, and may cause severe pain. The condition affects
individual of all ages but is particularly prevalent in children and
adolescents, when the emotional sequelae of facial deformities are
particularly severe.
Diagnosis
The diagnosis is clinically obvious, but biopsy is confirmatory. Biopsy must
be deep if non-caseating granulomas are not to be missed. Other histological
infilteration and intercellular oedema. Tuberculosis should be excluded. A
170
171
NUTRITIONAL DEFFICIENCY
Introduction
Development of Nutrient Deficiencies
Malnutrition and Periodontal Diseases
Oral Manifestations of Nutrient Deficiencies
Angular Stomatitis and Cheilosis
Altered Taste
Xerostomia
Changes in Tongue
Systemic Diseases Associated with Oral Manifestations and Nutrient
Deficiencies
BERI-BERI
PELLAGRA
SCURVY
RICKETS
OSTEOMALACIA
Patients with nutritional deficiency are common in the developing world but
are rarely seen elsewhere. Those most liable to be affected in the west are
the elderly, food cranks and alchohlics living on grossly unbalanced diet, or
patients with malabsorption. The relationship between nutrition and oral
health is multifaceted. Nutrition has both local and systemic impacts on the
oral cavity . While diet and eating patterns have a local effect on the teeth,
172
saliva and soft tissues, the systemic impact of nutrition also has considerable
implications and it too merits assessment as a component of comprehensive
care. The systemic effect is the impact of the nutrients consumed as they
assume their biological functions in relation to the development and
maintenance of the extra- and intra-oral structures and secretions . An
adequate supply of nutrients is essential to the growth, development, and
maintenance of tissues, effectiveness of the immune system, prevention of
cell damage and, in general, to increased resistance to many chronic, and
some infectious diseases . The oral cavity is often one of the first sites where
nutrient deficiencies can be clinically noted. Clinical manifestations of
nutrient deficiencies can have a significant impact on the function of the oral
cavity. Functional properties of the oral cavity include taste, salivation,
mastication, and swallowing food. Any alterations in the structure and
function of the oral cavity may compromise intake and contribute to the
development of a nutrient-deficiency state. When the associated oral
structures are affected, these alterations may be compounded even further,
leading to subsequent inadequate dietary intake and compromised nutrition
status.
Development of Nutrient Deficiencies
Nutrient deficiencies result from an imbalance of supply and demand, that
is, when the supply of nutrients is inadequate to meet the demands of the
body . The imbalance may result from one of three primary causes:
inadequate intake, impaired digestion and absorption, or increased losses. A
deficiency of any one nutrient may in turn contribute to subsequent
deficiencies of other nutrients. Clinical manifestations of deficiencies occur
once tissue stores are depleted. They may present as symptoms reported by
173
177
179
BERI-BERI
A prolonged gross deficiency of vitamin B1 i.e. thiamine causes beriberi.
There are three types of beriberi
-Wet beriberi
-Dry beriberi
-Infantile beriberi
Other diseases, which can be associated with it, are
- Wernicke's encephalopathy
-Peripheral neuritis
-Korsakoff's psychosis.
Wet beriberi
It is marked by cardiac dilation with four chamber enlargement, pallor and
flabbiness of myocardium.
Etiology
It is caused due to eating diets in which calories are derived from polished
rice. It is commonly seen in chronic alcoholics due to their poor nutrition in
general and also because alcohol interferes with intestinal absorption of
thiamine. It is often precipitated by infection, pregnancy and lactation.
Pathogenesis
Deficiency of thiamine will cause incomplete metabolism of glucose and
accumulation of pyruvic acid and lactic acid in tissue and body fluid which
leads to dilation of peripheral blood vessels and fluid may leak out through
capillaries, producing edema and high cardiac output.
Clinical features
There is pain in legs after walking due to accumulation of lactic acid. There
is tachycardia and increased blood pressure, cardiomegaly and palpitations.
There is also presence of sinus tachycardia and inverted T waves. Skin is
180
warm due to vasodilation. Edema may develop rapidly to involve leg, face
and trunk.
DRY BERI BERI
Clinical features
It is a peripheral neuropathy. In long-standing cases, there is degeneration
and demyelination of both sensory and motor nerve fibers resulting in severe
wasting of muscles. Blood pyruvate levels are normal.
Oral manifestations
There is hypersensitivity of oral mucosa. Pain in the tongue, teeth, jaws and
face.
Management
Complete rest. Thiamine 50 mg IM for 3 days then 10 mg 3 times daily by
oral route. Infantile beriberi is treated via mother's milk. Mothers should
receive 10,000 mcg twice daily. In addition, infants should be given
thiamine in doses of 10,000 to 20,000 mcg IM once in a day for 3days.
Wernicke's Encephalopathy
It is commonly seen in alcoholics with persistent vomiting.
There is a classical triad of ocular abnormalities, ataxia and confusion. There
are facial symmetrical areas of grayish discoloration. There is also bilateral
symmetrical ophthalmoplegia and ataxia. Histologically, there is
hypertrophy and hyperplasia of small blood vessels. Injection of thiamine
should be given 50 mg by slow intravenous injection followed by 50 mg
daily by oral route for a week.
Korsakoff's Psychosis
In it there is a predominant abnormality in mental function which is memory
defect. There is profound impairment of memory recall and new learning
ability.
181
Pellagra
Causes of pellagra are tryptophan deficiency,if insufficient tryptophan is
available for synthesis of niacin. Dietary deficiency of niacin. High dietary
levels of amino acid lucine antagonize the synthesis of NAD and
NADP(Nicotinamine Adenine Dinucleoide Phosphate). Chronic alcoholism,
diarrhoea and carcinoid syndrome can also cause pellagra.
Clinical features
It can be developed in 3 weeks with prodromal symptoms of loss of appetite,
vague gastrointestinal disturbances and numbness or burning in various
locations. It is called as disease of 3-Ds
-Dermatitis
-Diarrhea
-Dementia.
There is an erythema on skin resembling severe sunburns which appears
symmetrically overall parts of the body exposed to sunlight and especially
on the neck. Affected area is well demarcated from the normal. In acute
cases, skin lesions may produce vesiculation, cracking, exudation, crusting
with ulceration and secondary infection. In chronic cases, dermatitis occurs
as roughening and thickening of skin with brown pigmentation.
Alimentary tract is also affected it causes anorexia, nausea, dysphagia and
glossitis that precedes the skin lesions. Noninfective inflammation may
extend through the gastrointestinal tract. Diarrhea is caused by atrophy of
gastric epithelium followed by submucosal inflammation which is then
followed by ulceration.
182
Delirium is the most common mental disturbance in the acute form and
dementia in chronic cases. There is also loss of appetite, irritability and
burning sensation in different areas of the body.
Oral manifestations
Entire oral mucosa becomes fiery red and painful and salivation is profuse.
The epithelium of the entire tongue is desquamated. The filiform papillae are
most sensitive and disappear first, the fungiform papillae may become
enlarged. The tongue becomes red swollen and beefy and in animals the
deficiency leads to black tongue. In early stages, only the tip and margins of
the tongue are swollen and red. In advanced cases, the tongue losses all the
papillae and the reddening becomes intense.
In this stage, the tongue becomes so swollen that indentation from the teeth
are found along the borders of the tongue. The mouth is sore and shows
angular stomatitis, cheilitis. Tenderness, pain and ulceration begin at the
interdental papillae and spreads rapidly. Superimposed ANUG or Vincent's
infection involving the gingiva, tongue and mucosa is common.
Management
Niacin 10 mg or 10,000 mg per day. Vitamin B complex should be given and
if alcoholic patient should be adviced to stop the habit.
183
Scurvy
Prolonged deficiency of vitamin C may result in scurvy. It is characterized
by weakened blood vessels particularly micro vessels having least muscular
supports, defective synthesis of osteoid which is derivative of collagen and
impaired wound healing.
Pathogenesis
There is defective formation of collagen in connective tissues because of
failure of hydroxylation of proline to hydroxyproline which is a
characteristic amino acid of collagen. There is also increase permeability of
capillary (hemorrhage), anemia due to erythropoiesis and defective collagen
formation.
Clinical features
In infantile scurvy there is lassitude, anorexia, painful limbs and
enlargement of costochondral junction. Hair follicle rises above skin and
there are perifollicular hemorrhages, i.e. tiny points of bleeding occurring
around the orifice of hair follicles with heaping of keratin like material.
Hemorrhage may occur in the joint, into nerve sheath under the nails or
conjunctiva. Petechial hemorrhage occurs in buttocks, abdomen, legs, arms,
ankle and nail beds. Scorbutic child usually assumes a frog like position and
this may reflect as subperiosteal hemorrhage. Epistaxis, anemia and delayed
wound healing are common features. Edema of the limbs and face is a
frequent finding in severe ascorbic acid deficiency. It may lead to premature
aging, thyroid insufficiency and lower resistance to all infections.
184
Oral manifestations
It occurs chiefly involves gingiva and periodontal region. Interdental and
marginal gingiva is bright red, swollen, smooth with shiny surface producing
an appearance known as scurvy bud. In fully developed scurvy, the gingiva
becomes boggy, ulcerated and bleeds easily. Color of gingiva changes to
violaceous red. Typical fetid breath of the patient with fusospirochetal
stomatitis. In severe cases, hemorrhage and swelling of periodontal ligament
membrane occurs followed by loss of bone and loosening of teeth which are
exfoliated.
Histopathological features
Osteoblasts fail to form osteoid and cartilage cells of epiphyseal plate
continue to proliferate in normal fashion and salts are deposited in the matrix
between the columns of cartilage cells. The calcified matrix material is not
destroyed so the wide zone of calcified but nonossified matrix, called the
scorbutic lattice develops in the metaphysie.As the lattice increases in width
a more and more fragile zone develops, so that eventually complete fracture
of the spicule occurs with separation and deformity of the cartilage shaft
joint.
Laboratory features
Anemia in scurvy is mild to moderate but may be severe.It is usually
normocytic, normochromic, associated with leucopenia,
thrombocytopenia,reticulocytosis and normoblastic hyperplasia of the bone
marrow.
Management
Vitamin C 250mg three times daily can be given .
185
COLOR PLATE-13
NIACIN DEFFICIENCY,
loss of papillae and swollen red tongue
186
187
Clinical features
It occurs in infants and children. In the first 6 month of life, tetany,
convulsions are common manifestations due to hypocalcemia. The wrist and
ankles are typically swollen and the changes in bone are found in the
epiphyseal plates, metaphysis and the shaft. Localized area of thinning are
sometimes present in the skull, so that a finger can produce indentation. This
condition is called as 'craniotabes'. There is softening of posterior part of
the parietal bone, which may be first sign of the disease. Patients have a
short stature and deformed extremities. Children with rickets show bowing
of legs. Excess of osteoid produces frontal bossing and squared .appearance
to the head. Deformation of chest results from over growth cartilage or
osteoid tissue at the costochondrial junction producing 'rickety rosary'.
The weakened metaphyseal areas of the ribs are subject to pull of the
respiratory muscles and thus bend inwards creating anterior protrusion of the
sternum resulting in a pigeon breast deformity. The inward pull at the
margins of diaphragm creates Harrison's grooves, girdling the thoracic
cavity at the lower margin of the rib cage. When an ambulatory child
develops rickets, deformities are likely to affect the spine, pelvis and long
bones causing 'lumbar lordosis'.
Oral manifestations
Developmental abnormalities of dentin and enamel, delayed eruption and
malalignment of teeth.There is higher caries index in rickets as compared to
normal. There may be hypoplasia of enamel and enamel may be mottled,
yellow gray in color. There are large pulp chamber, high pulp horns and
delayed closure of root apices. The osteoid is so soft that teeth are displaced
leading to malocclusion of the teeth .
188
Radiographic features
The earliest and prominent manifestation is widening and fraying of
epiphysis of the long bones. Bowing is a characteristic deformity seen in the
weight bearing areas, fine trabeculae are reduced in number. Green stick
fractures will be noted in many cases in children.A thinning of jaw cortical
structure such as the inferior mandibular canal, the lamina dura and the
follicular walls of developing teeth has been described in rickets. The
trabeculae become reduced in number. In severe cases, jaws appear
completely radiolucent, so that teeth appear to be suspended in air.
If the disease occurs before 3 years of age, enamel hypoplasia is fairly
common. The pulp cavities of deciduous teeth are grossly enlarged.
The dentin is reduced to a thin margin separating the pulp cavity from
enamel and cementum. The density of existing dentin appears to be normal
and margins of pulp cavities are well and sharply defined. There is
narrowing of the periodontal ligament space.
Osteomalacia
It is also known as adult rickets and only flat bones and diaphyses of long
bones are affected. It is most commonly seen in postmenopausal females
with a history of low dietary calcium intake and little exposure to UV light.
Clinical features
It is seen in adults and pelvic deformities are commonly seen in females.
Remodeling of bone occur in the absence of adequate calcium resulting in
softening and distortion of the skeleton.The majority of patients have bone
pain and muscle weakness of varying severity. There is increased tendency
189
towards fracture, peculiar waddling or penguin gait. Tetany and green stick
bone fractures are also seen in some cases.
Oral manifestations
There is incidence of severe periodontitis in some cases of osteomalacia.
Radiographic features
A poorly calcified ribbon like zone extending into bone at approximately
right angles to the periosteal margin. They are partial or complete fracture
without displacement in which callus has been formed but there is no
calcium available to be deposited, thus healing process is not complete and
fracture remains apparent radioographically. It is also called as Looser's
zone. Osteoid tissue is formed in the defect but there is no calcium available
to be deposited in the osteoid. Pseudofracture of the jaws near the angle has
also been noted. Individual bony trabeculae may be sparse and unusually
coarse in intraoral periapical radiograph. The lamina dura may be thin or
absent in long-standing and severe cases of osteomalacia.
Biochemical changes
Elevation of serum alkaline phosphatase to three or more times its normal
levels. Serum phosphorus is low due to increased phosphorus excretion in
response to reduction of serum calcium. Serum calcium levels are usually on
the lower side.
Management of rickets and osteomalacia
Dietary enrichment of vitamin D in the form of milk. If tetany is present,
calcium gluconate IV Daily dose between 1000-2000 IU of vitamin D
190
191
RENAL DISORDERS
RENAL FAILURE
Acute Renal Failure
Chronic Renal Failure
Clinical Features
Oral Manifestations
Radiographic Features
Laboratory Diagnosis
RENAL FAILURE
Classification of renal failure is based on two criteria
The onset (acute versus chronic failure) and the location that precipitates
nephron destruction (prerenal, renal or instrinsic, and postrenal failure).
Chronic renal failure is a slow, irreversible, and progressive process that
occurs over a period of years whereas acute renal failure develops over a
period of days or weeks. The distinction between acute and chronic disease
is important, acute disease is usually reversible if managed appropriately
whereas chronic renal failure is a progressive and irreversible process that
leads to death in the absence of medical intervention.
ACUTE RENAL FAILURE
Acute renal failure (ARF) is a clinical syndrome characterized by a rapid
decline in kidney function over a period of days to weeks, leading to severe
azotemia (the building up of nitrogenous waste products in the blood).
Medications, surgery, pregnancy-related complications, and trauma are the
most common causes of ARF.
192
by
protein-restricted
diets.
Uremic
plasma
contains
Oral Manifestations
With impaired renal function, a decreased GFR, and the accumulation and
retention of various products of renal failure, the oral cavity may show a
variety of changes as the body progresses through an azotemic to a uremic
state. In studies of renal patients, up to 90% were found to have oral
symptoms of uremia. Some of the presenting signs are an ammonia-like taste
and smell, stomatitis, gingivitis, decreased salivary flow, xerostomia, and
parotitis. As renal failure develops, one of the early symptoms may be
a bad taste and odor in the mouth, particularly in the morning. This uremic
fetor, an ammoniacal odor, is typical of any uremic patient and is caused by
the high concentration of urea in the saliva and its subsequent breakdown to
ammonia. Salivary urea levels correlate well with the BUN levels, but no
fixed linear relationship exists.An acute rise in the BUN level may result in
uremic stomatitis, characterized by red mucosa covered with a thick exudate
194
COLOR PLATE-14
UREMIA,erthmatous
pseudomembranous uremic stomatitis
195
availability
and
improved
techniques
of
dialysis
and
occurs
due
to
impaired
calcium
absorption
and
Radiographic manifestations
Demineralization of bone is seen, loss of bony trabeculation which leads to
Ground-glass appearance. There is loss of lamina dura. Socket sclerosis,
pulpal narrowing and calcification. Giant cell lesions,brown tumors are seen
these are radiolucent lesions of hyperparathyroid harmone are calledbrown
tumors because they contain areas of old hemorrhage and appear brown on
clinical inspection. Arterial and oral calcifications is also evident .
Laboratory Investigations
Serum Chemistry
197
by
giving
hydroxylated
synthetic
analogue
of
vit-D.
198
INFECTIOUS DISEASES
BACTERIAL INFECTIONS
Tuberculosis
Leprosy
Tularemia
Tetanus
NOMA
VIRAL and FUNGAL INFECTIONS
Herpes simplex
Herpangioma
Chicken pox
Herpes zoster
Mumps
Actinomycosis
Mucoromycosis
TUBERCULOSIS
TB is the most common cause of death from a single microbial agent.TB is
responsible for almost 1 in 4 preventable deaths in the world.The World
Health Organization estimates that worldwide there are approximately 20
million active TB cases. Approximately 3 million people die each year from
TB, with 80% of this total occurring in developing countries.
199
infiltration
of
neutrophils,
macrophages,
and
these patients, microorganisms may spread throughout the body either (1) by
means of the blood, resulting in miliary tuberculosis,(2) via the respiratory
tissues, inducing a bronchopneumonia,or (3) through the gastrointestinal
tract as a result of the organisms being coughed up. In miliary tuberculosis,
foci of infection occur in distant organs and tissues but most frequently
develop in the meninges, lungs, liver, and renal cortex.
Reactivation Tuberculosis: Reactivation tuberculosis occurs in individuals
who have developed primary tuberculosis and who are asymptomatic, but
who still carry the bacteria within tubercles. These patients exhibit positive
tuberculin skin tests and thus demonstrate cellular immunity.Infection is
characterized by tubercle formation, caseation, fibrosis, and further
extension of the lesion.
Clinical Features
Patient may suffer episodes of fever and chills, easy fatigability and malaise.
There may be gradual loss of weight accommpanied by persistent cough
with or without hemoptysis. Local symptoms depend upon the tissue or
organs involved. Tubercular lymphadenitis may progress to acute abscess or
remain as granulomatous lesion. In any case, swelling of neck is present
which is tender, painful and often show inflammation of the overlying skin.
When abscess forms, it perforates and discharges pus.
Pulmonary tuberculosis
A persistence cough, hemoptysis abundant sputum is usual features of
pulmonary tubercuulosis. There is also evening rise in temperature of 0.5 to
2Fand night sweats. Scrofula is grandular form of disease resulting in
marked enrgement of the cervical lymph nodes with caseation and frequent
breakdown of the gland. Such tuberculosis infection is called as 'scrofula'
202
.The chronicity of the infection and the lack of marked pain or acute
inflammatory symptoms have resulted in the term 'cold abscess'.
They are relatively uncommon and seen in middle and older age groups. As
cleansing action of saliva and its antibacterial properties, in general, also
provide protection against tubrcle bacilli. Tongue is most commonly affected
followed by palate, lips, buccal mucosa and gingiva.Majority of oral lesions
are secondary to infection in some other parts of body.
Oral Manifestations
Oral manifestations of tuberculosis occur in approximately 3% of cases
involving long-standing pulmonary and/or systemic infection. 106,107 The
bacteria can infect oral tissues and lymph nodes (scrofula).Within the oral
cavity, lesions can occur in the soft tissues and supporting bone and in tooth
extraction sites, and may even affect the tongue and floor of the mouth
When reviewing this information, it becomes apparent that progression of
infection with tubercle bacilli to more severe disseminated stages occurs in
the absence of adequate cellular immunity to infection. Thus, the ability of
an infected individual to develop a dual immune response against M.
tuberculosis antigens greatly influences disease onset and progression. These
crucial protective responses are (1) acquired immunity to infection and (2)
development of tuberculin hypersensitivity.
Ulcer
The lesion may be preceded by an opalescent vesicle or nodule. A result of
caseation necrosis. It breaks down into an ulcer which is usually superficial
or deep and painful. It tends to increased slowly in size. In area of trauma
may be mistaken as traumatic ulcer or carcinoma. Ulcers are nonspecific in
their clinical presentation and for this reason they are overlooked by the
203
COLOR PLATE-15
PATHOGENESIS OF TUBERCULOSIS
MONTOUX TEST
204
206
Tuberculosis Vaccines
Bacille Calmette-Gurin (BCG), an attenuated strain of Mycobacterium
bovis, has been used for more than 80 years to protect humans against TB.
0.05 ml BCG vaccine is given intradermally to new born at the time of birth
at oter upper left arm or shoulder120.
paralysis
occurs
with
some
207
frequency
due
to
nerve
Oral Manifestations
The oral lesions that have been reported have generally consisted of small
tumor-like masses called lepromas, which develop on the tongue, lips or
hard palate. These nodules show a tendency to break down and ulcerate.
Gingival hyperplasia with loossening of the teeth has also been described,
but Reichart and his associates found that most of the gingival and
periodontal changes occurring in a group of 30 leprosy patients were
nonspecific. Skeletal changes involving face are rhinomaxillary changes
termed facies leprosa consists of atrophy of anterior nasal spine, atrophy and
recession of maxillary alveolar process confined to incisor region and
endonasal inflammation. Collapse of nose saddle nose is also seen.
Loss of lateral portion of eyebrow is common. Much latter, the skin of face
and forehead become thickened and corrugated (Leonine facies) and ear
lobe becomes pedunculous, nasal stiffness, epistaxis, hoarseness of voice is
seen
Histologic Features
The typical granulomatous nodule shows collection of epitheloid cells and
lymphocytes in a fibrous stroma. Langerhans type giant cells are variably
present. Vacuolated macrophages called lepra cells are scattered throughout
the lesions and often contain bacilli.
208
COLOR PLATE-16
209
Treatment
Specific
long
term
chemotherapy
is
initiated
upon
diagnosis.
NOMA
It is also called as Cancrum oris, gangrenous stomatitis. It is rapidly
spreading gangrene of oral and facial tissues occurring usually in debilitated
or nutritionally deficient person. Predisposing Factors are persons who are
undernourished. debilitated from infections such as diphtheria, dysentery,
measles, pneumonia, scarlet fever, syphilis, tuberculosis and blood
dyscrasias. It originates as a specific infection by Vincent's organisms.
Miscellaneous factors such as leukemia, sickle cell trait, stress and
chemotherapeutic agents can cause noma.
Clinical Features
It is seen chiefly in children, but can be found in adults in certain conditions
like in malnourished states. Common sites are areas of stagnation around
fixed bridge or crown. The commencement of gangrene is denoted by
blackening of skin. Small ulcers of gingival mucosa spread rapidly and
involve the sorrounding tissues of jaws, lips and cheeks by gangrenous
necrosis. Foul odour is present. Patients have high temperature during the
course of the disease, suffer secondary infection and may die from toxemia
or pneumonia.
Overlying skin is inflamed, edematous and finely necrotic which results in
formation of line of demarcation between healthy and dead tissue. In
advanced stage, there is blue black discoloration of the skin. As gangrenous
210
Management
Parenteral fluid should be given urgently to correct dehydration and
electrolyte balance. Blood transfusion helps in improving the clinical state of
the patient, who is usually anemic and toxic. The specific drug of choice is
penicillin although sulphonamides also yield good results. Reconstructive
surgery is necessary to lead near normal life.
NOMA, destructive
necrosis of oral and facial structures
211
SCARLET FEVER
Predominately occurs in children during winter months, caused by infection
with group-A streptococci of beta hemolytic type that elaborate erythrogenic
toxins.
Clinical Features
Incubation period is of 3 to 5 days. The desquamation of skin begins around
the middle of the second week after the onset. Patient exhibits severe
pharyngitis and tonsillitis, chills, fever and vomiting. Throat becomes highly
erythematous and exudation is common. There may be enlargement and
tenderness of regional lymph nodes.
Skin becomes bright scarlet to dusky red, skin rash may appear on the
second or third day of the illness. After 3 to 4 days, the rash fades. This rash
is due to toxic injury to the vascular endothelium which produces dilation of
the small blood vessels and consequent hyperemia.
Oral Manifestations
Stomatitis scarlatina accounts for the chief oral manifestation of scarlet
fever. Mucosa of palate may appear congested and throat is often fiery red.
Face, especially the temples and cheeks are flushed and red, but pale area of
circumoral pallor is often seen around the mouth. Tonsil and faucial pillars
are usually swollen and sometimes covered with grayish exudate.
Strawberry tongue exhibits white coating and fungiform papillae are
edematous and hyperemic, projecting above the surface as small red knobs
and it is called as 'strawberry tongue'.
Coating of tongue is soon lost, beginning at the tip and lateral margins and
the organ becomes deep red, glistening and smooth, except for swollen
212
perforation
of
palate.Osteomyelitis
and
involvement
of
the
DIPTHERIA
It is an acute contagious disease caused by gram +ve bacillus
Corynebacterium diphtheriae, also called as klebs loeffler bacillus.
Transmission
It is transmitted by droplet infection or direct contact.
213
Pathogenesis
The portal of entry is the upper respiratory tract and rarely skin, genitalia,
eye and middle ear. The bacilli settle on the mucous membrane or upper
respiratory tract and lead to inflammation and necrosis of mucosal cells. The
infection may spread to adjacent areas. In the primary invasive region, it
forms a thick, firm, leathery, blue white pseudomembrane composed of
bacteria, necrotic epithelium, macrophages and fibrin. A narrrow zone of
inflammation surrounding the area is seen.When the diphtheria bacteria
multiply in the local tissues, they produce powerful exotoxins. This exotoxin
diffuses through the body through a hematogenous route. Heart, muscle,
kidney, peripheral nerves and adrenal glands are thus involved. Death may
be caused by heart failure, airway obstruction which is caused by edema or
by the effect of toxin.
Clinical Features
It occurs most frequently in children, during the fall and winter months
Listlessness, malaise, headache, fever and occasional vomiting.
Within a short time, patient complains of sore throat. Mild redness and
edema of pharynx with cervical lymphadenopathy. There may be swelling of
the neck, called as bull neck. Nasal regurgitation of liquids during drinking.
Larynx is edematous and is covered by pseudomembrane.There is
generalized polyneuritis with weakness, paresthesia may follow in the next
10 to 14 days.
Oral Manifestations
There is formation of patchy 'diphtheritic membrane' which begins on tonsils
and enlarges, becoming confluent over the surface. It is thick and grayish in
color. It tends to adhere and leave a raw bleeding surface on removal. Soft
palate shows temporary paralyses usually during 3rd and 5th weeks ofthe
214
215
TULAREMIA
It is caused by gram-negative bacillus Francisella tularensis. It is contacted
through infected rabbits, muskrats, ground squirrels and other wild germ,
particularly of rodent family.
Types
-Cutaneous
-Ophthalmic
-Pleuropulmonary
-Oral
-Abdominal
Clinical Features
Incubation period is up to seven days. Patient usually suffers a sudden
headache, nausea, bony pain, profuse sweat, vomiting, chills and fever.
A single cut or sore on the skin develops into a suppurative ulcer.
Lymph nodes become swollen and painful and the lymph nodes are
remarkably enlarged.Eyes are also involved with conjunctivitis developing
through localization of disease in the conjunctival sac.
Complications
Tularemic pneumonia and pleuritis are complications of this disease.
Oral Manifestations
Primary infection of the mouth usually occurs from eating infected meat.
It is common on soft palate, tongue gingiva and angle of mouth.
It is usually accompanied by severe pain. Ulcer is shallow with whitish
fibrinous pseudomembrane, but may extend more deeply with superinfection
of Vincent's organism. Generalized stomatitis may develop. Single nodular
masses eventually develops into abscess. The tonsil, posterior pharyngeal
wall, soft palate, base of the tongue and buccal mucosa may be covered by a
216
217
Types
Local tetanus
Cephalic form
Generalized form
Neonatal tetanus
Chronic tetanus
Clinical Features
It is more common in young males during their accident prone years.
Local tetanus
It is characterized by muscle spasm near the site of entry of bacilli. In some
cases it may proceed to generalized form. Mortality rate is less than 1
percent in this form.
Cephalic form
In some cases bacilli are introduced through the wounds in the head and
neck region. Cranial nerve palsy occurs with cranial nerve, most commonly
the 7th cranial nerve.
Generalized form
Pain and stiffness in jaw, neck muscles with muscle rigidity producing
trismus and dysphagia. Rigidity of facial muscles may also occur producing
the typical risus sardonicus. Sometimes, entire body may be affected with
contraction of all groups of somatic muscles leading to characteristic
opisthotonos. Reflex spasm frequently develops after stimuli. Acute trismus
may stimulate acute oral infection, trauma, temporomandibular dysfunction
and even hysteria. The temperature is usually raised due to increase
metabolic rate. When spasm of intercostal pharyngeal and diaphragmatic
muscle occurs adequate ventilation becomes impossible and the resultant
anoxia causes death. Death in patients with tetanus is generally related to
218
tetanus
immunoglobulin.
1,000,000
units
of
penicillin
Vaccination
Tetanus toxoid(TT) vaccine is administered every 10 years and as early as
possible in pregnancy 0.5ml IM at upper arm region.120
219
VIRAL INFECTIONS
Herpes simplex
Herpangioma
Chicken pox
Herpes zoster
Mumps
220
The usual ganglion involved is the trigeminal for HSV-l and lumbosacral, for
HSV-2.
Transmission
It occurs during close personal contact.
Primary infection of newborn is believed to be caused by vaginal secretions
during birth, which results in viremia and disseminated infection of brain,
liver, adrenals and lungs.
Dentist may experience primary lesion of fingers from contact with lesions
of the mouth or saliva of the patients who are asymptomatic carriers of HSV,
called as herpetic whitlow.
Clinical Features
Incubation period is 5 to 7 days, but may range from 2 to 12 days. It
develops in both, children and young adults. Prodromal symptoms precede
local lesion by 1 to 2 days and it includes fever, headache, malaise, nausea,
vomiting and within a few days, mouth becomes painful. There is also
irritability, pain upon swallowing and regional lymphadenopathy. Small
vesicles, which are thin walled, surrounded by inflammatory base are
formed. They quickly rupture leaving small, shallow, oval shaped discrete
ulcers. The base of the ulcer is covered with grayish white or yellow plaque.
The margins of the sloughed lesions are uneven and are accentuated by
bright red rimmed, well demarcated, inflammatory halos. The individual
ulcer differs in size from 2 to 6 mm. As the disease progresses several
lesions may coalesce, forming larger, irregular lesions. Lips in severe cases,
excoriation involving the lips may become hemorrhagic and matted with
serosanguinous fibrin like exudate and parting of the lips during mastication
and speech, may become extremely painful and difficult.Acute marginal
gingivitis, entire gingiva is edematous and swollen and small gingival ulcers
221
are
seen.
Posterior
pharynx
reveals
inflammation,
cervical
and
dysuria
may
develop,
espeecially
in
women.
Regional
223
Histopathological Features
Intraepithelial blisters filled with fluid. Intranuclear inclusions, Lipschutz
bodies which are eosinophilic, civoid, homogenous structure within the
nucleus, tend to displace the nucleolus and nuclear chromatin peripherally.
The displacement of chromatin often produces a peri-inclusion halo. When
vesicle rupture, surface of the lesion is covered by exudate made up of fibrin
polymorphoonuclear leukocytes and degenerated cells.
Diagnosis
Negative past history of recurrent herpes labialis and a positive history of
close contact with a patient with primary or recurrent herpes is helpful in
making the diagnosis.Patient is easily diagnosed as having primary herpetic
gingivostomatitis, if he/she presents with typical clinical features of
generalized symptoms followed by eruption of oral vesicles and acute
marginal gingivitis and does not have history of recurrent herpes.
HSV isolation and neutralization of virus in tissue culture is most positive
method of identification. Rabbit kidney and human amnion are sensitive to
HSV. Antibody titer antibodies to HSV appear in a week and react peak in 3
weeks.
Treatment
Symptomatic
Topical anesthetics like lignocaine, dyclonine hydrochloride 0.5 percent,
benzocaine hydroochloride are used. Topical anti-infective agents to prevent
secondary infection are 0.2 percent chlorohexidine gluconate, tetracycline
mouthwash.Solution of diphenylhydramine hydrochloride (Benadryl) 5 mg
mixed with equal amount of milk of magnesia. Fluid to maintain proper
hydration and electrolyte balance. Maintenance of oral hygiene.
224
Specific Treatment
Acyclovir inhibits DNA replication in HSV infected cells reducing the
duration of illness but with few side effects. The optimum oral dosage of
acyclovir is 1,000 to 1600 mg daily, for 7 to 10 days. Topical acyclovir is not
useful for treating intraoral lesions and may not be effective for lesions on
lips. Other antiviral agents have been utilized with success. Idoxiurdine,
cytosine arabinoside and adenine arabinoside have been used systemically.
These should only be used in special cases of HSV infection associated with
immune deficiency.
Recurrent or secondary herpetic infection
Recurrent infections are limited to localized portions of skin and mucous
membrane.
Types
-Recurrent herpes labialis (RHL).
-Recurrent intraoral herpes simplex infection (RIH).
Precipitating factors
Surgery involving trigeminal ganglion as it remains latent in trigeminal
ganglion,trauma to lips, fever, emotional upset, upper respiratory tract
infection, sunburns, fatigue, menstruation and pregnancy are ptrecipitating
factors.
Immunity
Low serum IgA, decreased cell mediated immunity, decreased anti herpes
activity and depression of ADCC (antibody dependent cellular cytotoxicity)
and interlukin-2, caused by prostaglandin release in skin. The virus once
introduced into the body, appear to reside within the regional ganglia. When
reactivation is triggered, they spread along the nerves to different sites on
225
oral mucosa and skin, destroy the epithelial cells and induce the typical
inflammatory response with characteristic lesions of recurrent infection.
Clinical features
If it occurs on lip, it is called as recurrent herpes labialis. If occurs
intraorally it is called as recurrent intraoral herpes infection. Recurrent
herpes simplex infection may occur at widely varying intervals, from nearly
every month in some patients to only about once a year or even less in
others. Prodromal symptoms in either location, lesion is preceded by tingling
and burning sensation and feeling of tautness, swelling or slight soreness
subsequent development of vesicle.It is accompanied by edema at the site of
the lesion, followed by formation of clusters of small vesicles. It range from
1 to 3 mm in diameter, to 1 to 2 cm. But sometimes, it is large enough to
cause disfigurement. These gray or white vesicles rupture quickly leaving
small red ulcerations, sometimes with slightly erythematous halo on lip
covered by brownish crust on lips.
Recurrent intraoral herpes
In RIH vesicles break rapidly to form small red ulceration, sometimes with
slight erythematous halo.Cluster of small vesicles or ulcers 1 to 2 mm in
diameter are commonly found on gingiva, palate and alveolar region.
The lesions gradually heal within 7 to 10 days and leave no scars.
Management
Minimized obvious trigger.
Oral acyclovir.
Topical use of carbon oxolone useful in herpetic gingivostomatitis.
226
COLOR PLATE-17
HERPETIC GINGIVOSTOMATITIS
burst vesicles on labial mucosa
227
transmitted to animals, e.g., the rabbit corrnea, as can the simplex virus. The
most important feature is its unilateral occurrence along the infected nerve
tract.The triggering factors initiating the onset of an attack of herpes zoster
are varied and may include trauma, development of malignancy or tumor
involvement
of
dorsal
immunosuppressive
root
therapy.
ganglia,
It
is
local
a
x-ray
common
radiation
or
infection
in
229
COLOR PLATE-18
Herpes zoster
ULCERATION ON GINGIVA
230
Diagnosis
Herpes zoster can frequently be recognized by the characteristic distribution
of the lesions, although there may be a similarity to the lesions of herpes
simplex infection. Skin lesions and oral lesions in particular may be easily
identified as viral diseases by cytologic smears and the finding of
characteristic nucleated giant cells (Tzanck test) and intranuclear inclusions.
However, this does not differentiate between herpes zoster and
herpes
Treatment
The newer anti-viral drugs are now under intensive clinical testing for
potential effectiveness in treatment of herpes zoster. The preliminary results
appear very promising.
Postherpetic neuralgia
Lesions limited to the course of a sensory nerve.It is caused by reactivation
of latent varicella zoster virus infection that results in pain and vesicle
formation along the course of affected nerve.If Longer course of diseases
is present, if pain persists longer then a month it is called post zoster
neuralgia. PHN may occur at any age but major risk factor is increasing age.
To control postherpetic neuralgia, prednisone 40 to 60 mg daily for 1 to 2
weeks. Steroid injection can be given to patient with age more than 60 years,
for the treatment of post-herpetic neuralgia.
Antidepressants like amitriptyline and other tricyclic antidepressant have
been used to minimize painful sequelae of this infection. Sympathetic nerve
block and chemical and surgical neurolysis is also useful in some cases.
231
HERPANGINA
It is also called as aphthous pharyngitis, vesicular pharyngitis. Frequently
occurs in epidemic,with highest frequency from June to October. It appears
to be transmitted from one person to another through contact.
Clinical features
Majority affected are young children aged 3 to 10 years. Incubation period is
of 2 to 10 days. Generalized symptoms are of fever, chills, headache,
anorexia, abdominal pain and sometimes vomiting. Sore throat, dysphagia
and occasionally, sore mouth can occur. It occurs on posterior pharynx,
tonsi1, faucial pillars and soft palate. Lesion starts as punctuate macule
which evolves into papules and vesicles. Within 24 to 48 hours, vesicles get
ruptured forming small 1 to 2 mm ulcers. Base and margins of ulcers show a
gray base and inflamed periphery. They generally heal without treatment in 1
week.
Laboratory diagnosis
No ballooning degeneration seen which is helpful to distinguish herpangina
from herpes simplex and herpes zoster.
Difference between Herpangina and primary HSV is that Herpangina occur
in epidemic, HSV does not. Clinical manifestations of herpangina are
generally milder than HSV infection. Lesions of herpangina occur in
pharynx and posterior portions of oral mucosa. Herpangina does not cause
generalized acute, marginal gingivitis. Lesions of herpangina are smaller
than HSV.
Management
Self limiting and supportive treatment by proper hydration and topical
anesthetic, when eating or swallowing is difficult .
232
MUMPS
(Epidemic Parotitis)
Mumps is an acute contagious viral infection characterized chiefly by
unilateral or bilateral swelling of the salivary glands, usually parotid. The
submaxillary and sublingual are occasionally involved, but seldom without
parotid involvement also. Occasionally internal organs rather than the
salivary glands are involved. Although it is usually a disease of childhood,
mumps may also affect adult in such cases there is a greater tendency for
complications to develop. Mumps has incubation period of two to three
weeks.
Clinical Features
The disease is usually preceded by the onset of headache, chills moderate
fever, vomiting, and pain below the ear. These symptoms are followed by
firm some what rubbery or elastic swelling of the glands, frequently
elevating the ear, which usually lasts about one week. This salivary
involvement produces pain upon mastication.Bilateral parotid involvement
occurs in 70 per cent of the cases.
It is of interest to note that the virus of epidemic parotitis is present in the
affected persons. For this reason, droplet dissemination and infection are
common. It is also reported that the papilla of the opening of parotid duct on
the buccal mucosa is often puffy and reddened.
Complications
Other organs of the body may be affected as a complication of the disease.
These include the testes, ovaries, pancreatic gland, mammary glands and
occasionally the prostrate, epididymis and heart. When mumps involve the
adult male orchitis is a great danger ensues in approximately 20 per cent
cases. This orchitis is usually unilateral occasionally complete sterility
233
deafness
and
mastitis
are
also
occasional
Nonspecific mumps
There are several "nonspecific" conditions chracterized by enlargement of
one or more major salivary glands that are not related etiologically to
epidemic parotitis, or true mumps but yet may produce considerable
difficulty in diagnosis and differential separation from true mumps of viral
origin. Although not all of these are of specific microbial origin, some of the
more common conditions will be discussed here because of clinical and
occasional microscopic resemblance to epidemic parotitis, or mumps. These
include (1) chronic nonspecific sialadenitis (2) acute postoperative parotitis
(surgical mumps or
This condition been reviewed by King and Koerner, and was noted to be
most common in adults, particularly males. The most frequent cause of
chronic sialadenitis is the occurrence of salivary duct calculi
with
accompanied by severe pain and rapid swelling of the parotid gland. The
overlying skin may be reddened, and the associated edema may involve the
cheek, periorbital area and neck. Trismus is present, as is a low-grade fever
with headache, malaise and leukocytosis. A purulent discharge may be
expressed from the parotid duct by digital pressure along the duct toward its
orifice. Treatment of this condition is generally the administration of
antibiotics.
NUTRITIONAL MUMPS
Numerous investigators have reported a chronic, asymptomatic, bilateral
enlargement of the parotid or submaxillary glands occurring endemically in
populations suffering from malnutrition. The dietary factors specifically
involved have not been identified, but the lesions occur most frequently in
patients
with
multiple
signs
of
nutritional
deficiency
such
as
236
Sjogren's
237
MEASLES
It is also called as Rubeola or morbilli. It is an acute contagious
dermatotropic viral infection, primarily affecting children and occurs many
times in epidemic form.
Transmission
Spread of disease occurs by direct contact with a person or by droplet
infection, the portal of entry being the respiratory tract.
Clinical Features
Incubation period is 8 to 10 days. Onset of fever, malaise, cough,
conjunctivitis, photophobia, lacrimation and eruptive lesions of skin and oral
mucosa. Otitis media and sore throat can occur. Skin eruption begins on
face, in the hair line and behind the ear and spread to neck, chest, back and
extremities. It appears as tiny red macules or papules which enlarge and
coalesce to form blotchy discolored irregular lesions, which blanch on
pressure. Fade away in 4 to 5 days with fine desquamation .
Oral Manifestations
Oral lesions precede 2 to 3 days before cutaneous rash and are
pathognomonic of this disease. The most common site is on buccal mucosa.
Intraoral lesions are called as Koplik's spots and occur in 97 percent of
cases.They are small, irregularly shaped flecks which appear as bluish white
specks surrounded by bright red margins.
Treatment
The patient should be isolated if possible and treated with antiviral drugs.
Vitamin A should be given.
238
PREVENTION
Active Immunization
One injection of live attenuated measles virus 0.5ml should be given
subcutaneously in children over one year at 12-15 months.120
Passive Immunization
Human immunoglobulin given intramuscularly is recommended for
prevention and attenuation of measles, particularily for contact under 18
months of age and for debilitated children. The dose is 250mg for children
under 1 year and 500mg for those over this age.
Complications
Disease lowers general body resistance and for this reason it often leads to
bronchial asthma, encephalitis, otitis media, NOMA, Hodgkins Lymphoma.
CHICKEN POX
(Varicella)
Chicken pox is an acute viral disease,ubiquitous,extremely contagious
disease usually occurring in children, and is characterized by an
exanthematous vesicular rash.It is most common in winters and spring
months.
Etiology
Varicalla zoster virus is DNA virus which causes two distinct lesion
chickenpox, a primary lesion and herpes zoster.The incubation period is
approximately two weeks. It has been stated by Sabin that the virus causing
this disease is one of the most contagious and sooner or later infects
everyone in the world. It rather closely resembles smallpox,but is far less
severe. The virus is the same as that which causes herpes zoster, and the
lesions of two diseases have many features in common.The transmission is
239
by airborne droplets or contact with infected lesions, with the portable portal
of entry being the respiratory tract
Clinical Features
The disease is characterized prodromal occurrence of headache,
nasopharyngitis, and anorexia, followed by macuulopapular or vesicular
eruptions of the skin and low-grade fever. These eruptions usually begin on
the trunk and spread to involve the face and extremities. They occur in
successive crops so that many vesicles in different stages of formation or
resorption may be found. The lesions of the skin eventually rupture, form a
superficial crust and heal by desquamation. The disease runs its clinical
course in a week to ten days, seldom leaving any after-effects. Occasionally,
secondary infection of the vesicles results in the formation of pustules which
may leave small pitting scars upon healing.
Oral Manifestations
Small blister-like lesions occasionally involve the oral mucosa, chiefly the
buccal mucosa, tongue, gingiva and palate, as well as the mucosa of the
pharynx . The mucosal lesions, initially slightly raised vesicles with a
surrounding erythema, rupture soon after formation and form small eroded
ulcers with a red margin, closely resembling aphthous lesions. These lesions
are not particcularly painful.
Complications
Complications are not commmon, and the mortality rate is extremely low.
Encephalitis or pneumonia occasionally occurs. However, children with
chronic diseases, those receiving cortisone therapy or those with
malignancies and receiving chemotherapy are prone to develop a severe or
even fatal form of the disease.
240
FUNGAL INFECTIONS
Mucormycosis
Actinomycosis
MUCORMYCOSIS
It is caused by saprophyte fungus. More common in patients with decreased
resistance due to disease like diabetes, tuberculosis, renal failure, leukemia,
cirrhosis and in severe burn cases.
TYPES
Superficial: It involves external ear, fingernails and skin.
Visceral: Pulmonary, gastrointestinal, rhonocerebral or rhinomaxilllary.
Clinical Features
Rhinomaxillary form begins with inhalation of fungus by susceptible
individual.Infection usually arises in lateral wall of nose and maxillary
sinus; may rapidly spread by arterial invasion to involve the orbit, palate,
maxillary alveolus and ultimately the cavernous sinus and brain through
hematogenous spread and may cause death. Ptosis, proptosis, fever, swelling
of cheek and paresthesia of face can occur. Intracranial involvement may be
heralded by cranial neuropathy, especially of the trigeminal and facial nerve.
There is increased lethargy, progressive neurologic deficit and ultimately
death.
Fungus invades artery to cause fibrosis and ischemia. There is appearance of
a reddish black nasal turbinate and septum. Nasal discharge caused by
necrosis of nasal turbinate.
241
Oral Manifestations
Ulceration of palate, due to necrosis and invasion of palatal vessels resulting
in perforation of palate. Ulcer may be seen on gingivae, lip and alveolar
bone.It is large and deep, causing denudation of underlying bone.
Radiographic Features
Paranasal sinus may reveal mucoperiosteal thickenning of the involved
sinus. With disease progression, there is increased nodularity and soft tissue
thickening, usually mimics a tumor on radiographical examination.
CT scan is most helpful for detecting the degree of bone destruction and for
evaluating disease extension into the orbit and brain.
Histopathological Findings
The tissue involved by mucormycosis shows necrosis and chronic
inflammatory infiltrate. The vessels in the area may be thrombosed with
organisms in the lumen. Culture studies are essential to identify the order
family and genus of fungus. The organism appears as large, nonseptate
hyphae with branching at obtuse angle.
Management
Surgical debridement is the treatment of choice.
Systemic amphotericin in dosage of 0.15-0.2mg/kg/day once a day.
Control of predisposing factors such as diabetes.
Elimination of secondary infections and symptomatic relief.
242
ACTINOMYCOSIS
It is a chronic granulomatous suppurative and fibrous type of disease caused
by anaerobic, gram +ve nonacid-fast bacteria. Most common are
Actinomyces israelii, A nasalundi, A viscosus and A odontolyticus. The
organism is considered to be transitional form between bacteria and fungi.
The term Actinomyces was given by Harz to refer the ray like appearance of
the organism in the granule. The breach in the continuity of mucosa caused
either by trauma or surgery, is the prerequisite for majority of actinomycosis
infections.
Classification
Cervicofacial
Abdominal
Pulmonary
Cutaneous
Types
Central: Here, the infection is from the tooth or its membrane and is
accompanied by radiographic changes.
Peripheral:The peripheral types originate in the soft tissues and do not
involve bone.
Predisposing Factors
Trauma
Presence of carious teeth
Secondary bacterial invasion
Hypersensitivity reaction
243
Cervicofacial form
It is most common type of actinomycosis and is commonly seen in adult
males. Cervicofacial actinomycosis infections are endogenous in origin and
occur when dental plaque, calculus or gingival debris contaminate the
relatively deep wounds around the mouth.The classical signs are chronic low
grade persistence infection. Submandibular region is the most frequent site
of infection.
244
Pulmonary form
It produces findings such as fever and chills, accompanied by a productive
cough and pleural pain. Pleural invasion resulting in empyema and there
may be formation of sinus.
Subcutaneous form
Infection results from traumatic transplantation of organism, usually due to
human bites. Lesion seen as subcutaneous swelling which enlarges slowly,
softens and ruptures through the sinuses. Occasionally, these lesions burrow
through deeper tissue and invade bones.
Oral Manifestations
Organism may enter the tissue through oral mucous membrane and may
either remain localized in the adjacent soft tissue or spread to involve
salivary glands. bone or skin of face and neck. It produces swelling and
induration of tissue. It may develop into one or more abscesses which tend
to discharge upon the skin surface liberating pus which contains typical
sulfur granules. There may be non healing tooth socket, exuberant
granulation tissue and periosteal thickening of alveolus. Skin overlying
abscess is purple red and indurate or fluctuant. It is common for sinus,
through which the abscess has drained, to heal but due to chronicity, new
abscesses are formed and perforate through skin surface. There is
disfigurement of face. Infection may involve maxilla and mandible.
Periapical granuloma formation is also seen in some cases.On tongue the
lesion is a painful nodule which eventually ulcerates. Untreated cases may
reach to the point where the tongue may become fixed.
245
Radiographic Features
The radiographic appearance may resemble with apical rarefying osteitis.
It may appear as an area of bone destruction, which resembles a dental cyst,
with a well defined area of radiolucency with cortical lining of dense bone.
Lamina dura is deficient at the apex of tooth. Scattered area of bone
destruction, separated from one another by normal or sclerosed bone, is
another manifestation. The persistence radiolucency of tooth socket with an
increased density of adjacent bone may be the first sign of disease.
Histopathological Features
Ray fungus appearance, round or lobulated colony meshwork of filaments
stain with hematoxylin and peripheral club shaped ends of filaments stain
with eosin.
Management
Actinomyces infection produces a reactive inflammatory response which
causes an area of necrosis and scar tissue around the abscess. This results in
decrease in the vascular supply to the affected region and hence makes
penetration of antibiotics difficult. Therefore, two-fold therapy including
antibiotics and surgery is necessary. The lesion should be surgically removed
and the surrounding area should be thoroughly debride. Following surgical
intervention, antibiotics therapy should be continued. The antibiotic of
choice is penicillin which should be given 3 to 4 million IV, every 4 hours
for 2 to 4 weeks. This should be followed by 0.5 to 12 gm of penicillin, four
times a day for 4 to 6 weeks. In patients allergic to penicillin, tetracycline
orally 500 mg given four times a day for 6 months. Other agents used in the
246
247
SYPHILIS
It is also called as Lues. Veneral disease caused by spirochetes T. Pallidum.G
+ motile, microaerophilic spirochete, pathogenic to humans, can be
demonstrated by dark field microscope.
Classification
1.Acquired 2.Congenital
ACQUIRED SYPHILIS
Contacted primarily as venereal disease due to sexual intercourse with
infected partner
1. Primary
2. Secondary
3. Tertiary
248
CONGENITAL SYPHILIS
Early syphilis
Primary syphilis, secondary syphilis and the early latent phase of the disease
are grouped as early syphilis. Early syphilis may last up to two years and is
infectious.
Late syphilis
While late latent and tertiary are grouped as late syphilis. Late syphilis is
locally destructive and non-infectious.
Epidemiology
Decreased incidence after introduction of penicillin in late 1940s. Patient
infected with Treponema pallidum and HIV may exhibit a malignant form of
syphilis with slow development of standard serologic response to syphilis.
The tertiary manifestations lead to considerable morbidity and mortality.
PRIMARY SYPHILIS
in the primary stage, a lesion known as chancre develops at the site of
inoculation approximately 3-90 days after contact with infection.
Clinical features
Incubation period-lesions develop at the site of inoculation approximately 3
weeks after contact with infection.
SITE
It occurs most frequently on penis in males and vulva or cervix in females.
Recently, occurrence on extragenital sites have increased as a result of
249
Lymph nodes
Regional lymph nodes become firm enlarged, rubbery in consistency and
non-tender.
Signs:The combination of indurated ulcer on appropriate mucosal surface
and enlarged, nonntender, regional lymph nodes which are painless, discrete
and rubbery in consistency-constitute the classic signs of primary syphilis.
Oral manifestations
Site:Oral lesions of primary syphilis are rare and occur at the site of entry of
Treponema. Chancre has been described on lips, in males (upper lip) and in
females (lower lip), oral mucosa,lateral surface of tongue, soft palate,
tonsillar area, pharyngeal lesion and gingiva.
Transmission:It can occur during kissing as a consequence of sexual practice
among homosexual and heterosexuals or by contact with objects such as
mouthpiece of musical instruments and medical or dental instruments.
250
SECONDARY SYPHILIS
Secondary or metastatic stage, usually commencing about 6 weeks after
primary lesion is usually characterized by diffuse eruption of skin and
mucous membrane. In contrast to lesion in primary stage, lesions of
secondary stage are typically multiple.
Clinical features
Organisms proliferate and spread by the way of bloodstream to produce
lesions elsewhere. It usually appears within 3 to 6 weeks after primary
lesion.When appear on skin, they manifest as fine macular papular rash,
sometimes accompanied by alopecia.Circinate lesions on face are
characteristic of secondary syphilis.The lesions either resolve completely(or
leave residual areas of hypo or hyperpigmenntation. Fever and generalized
lymphadenopathy, which is painless, discrete and nonadherent to the
251
Oral manifestations
Mucus patches: Mucus membrane analogue of papular or macular skin
eruptions. It is found on tongue, buccal mucosa, tonsillar and pharyngeal
region and lips. It appears as slightly raised grayish white lesions surrounded
by erythematous base. They are covered by grayish-white membrane.
Trauma results in raw bleeding surface.
Snail track ulcers: Confluence and coalescence of these glistening mucous
patches gives rise to the so called snail track ulcers. It is often painless but
mild to moderately painful.
Split papule: Split papule is a raised papular lesion developed at the
commissure of lip and with a fissure separating the upper lip portion from
lower lip portion. They are called as split papules as they are cracked in the
middle giving a 'split pea appearance' . They are highly infectious.
252
Condyloma latum: They are flat silver gray wart like papule, sometimes
having ulcerated surface. They are painless. Regional lymphadenopathy is
usually present. It may occur at any age from the third year upto the patient's
life.
Forms: In tertiary syphilis, 1/3rd develop benign or gummatous form, 1/3rd
cardiovascular form and '1/3rd neurosyphilis, i.e. general paresis and tabes
dorsalis.
Gumma
Gumma is due to a chronic destructive granulomatous process which occurs
anywhere in the body. Gumma is the result of hypersensitivity reaction
between hyperergic host and treponema. There are two types of gumma, i.e.
central and cortical. The characteristic gumma is a chronic granulomatous
and usually localized lesion.Which later ulcerates and which may be
nodular. Punched out ulcer with vertical walls and dull red granulomatous
base is the typical clinical feature of ulcerative gummatous lesion.
Cutaneous lesions heal slowly and leave behind tissue paper like scars.
Single cerebral gumma may produce symptoms suggestive of brain tumor
Neurosyphilis
It occurs due to obliteration of small vessel artery involving vasa vasorum of
aorta other large vessels of the central nervous system (neurosyphilis).There
is saddle deformities of nose. Neurosyphilis is manifested as tabes dorsalis
and general paresis. Tabes dorsalis is the syphilitic involvement of dorsal
column of spinal cord and dorsal root ganglion. General paresis is syphilitic
involvement of cerebral tissue.
Tabes dorsalis
Patient looses the positional sense of his lower extremities and walks with a
slapping step. Burning and pricking sensation of the extremities, paresthesia,
253
254
Gumma may manifest as solitary, deep, punched out mucosal ulcer, pale,
raised, nodular mass in the midline of the palate which ulcerates and rapidly
progresses to the zone of necrosis. It may cause perforation of palatal vault.
COLOR PLATE -20
PRIMARY SYPILIS
SECONDARY SYPHILIS
255
Lesion is sharply demarcated and the necrotic tissue at the base of the ulcer
may slough away leaving punched-out defects. Breathing and swallowing
difficulty may be encountered by the patient. Numerous small healed
gummata in tongue results in series of nodules or scars in deeper areas of the
organ, giving the tongue an upholstered or tufted appearance.Complete
atrophy of papillary coating and firm fibrous texture seen in syphilitic bald
tongue is also referred to as chronic superficial interstitial glossitis. Loss of
papillae is probably due to endarteritis leading to circulatory deficiency of
lingual vasculature. It has been labeled as precancerous because of its
predilection to undergo carcinomatous transforrmation.
CONGENITAL SYPHILIS
Congenital syphilis is transmitted to offspring only by infected mother and is
not inherited. Though congenital syphilis is preventable, it still continues to
be a major problem in many countries.it is recognized that if antibiotic
treatment with antibiotics is begun in infected pregnant women before fourth
month of pregnancy offspring of these mother wil be free of disease.Three
possibilities can occur if mother is infected one third of pregnancies result in
abortion that is, still birth Infantile and one third deliver normal children
256
syphilis.
Early Congenital Syphilis: Maculopapular rash, Condylomata lata, Mucous
patches, Fissures around mouth, nose and anus. Rhinitis with nasal discharge
hepatosplenomegaly,osteochondritis/periostitis,generalizedlymphadenopath,
choroiditis meningitis, anemia / thrombocytopenia
Late Congenital Syphilis: Also called as Benign tertiary syphilis Periostitis
paroxysmal cold hemoglobinuria, neurosyphilis, 8th nerve deafness,
interstitial keratitis, Cluttons joints (Painless effusion into knee joints),
Stigmata of Congenital Syphilis: Hutchinsors incisors, mulbery molars,
high arched palate, maxillary hypoplasia, saddle nose, rhagades (radiating
scars around mouth, nose and anus) salt and pepper scars on retina, corneal
scars, sabre tibia (from periostitis) bossing of frontal and parietal bone.
Radiographic Features of Syphilis: Bones are not known to be involved in
the primary stage. Bone involvement usually occurs in tertiary stage and in
some cases, in secondary stages. Palatal perforation is seen in some cases.
Diagnosis
Examine exudates under dark field microscope for spirochetes.
Immunological tests:
1.REAGIN Ab test: These tests use the lipoidal or cardiolipin Ag and are
known as standard tests for syphilis STS. The first reagin Ab tests was the
WASSERMAN COMPLEMENT FIXATION TEST 1906 which originally
used watery extract of liver of syphilitic fetus as antigen.This was latter
substituted by an alchohlic extract of ox heart tissue, to which lecithin and
cholestrol are added now called as VDRL test,VDRL Ag(cardiolipin,
257
GONORRHEA
It is primarily an infection of genitourinary tract mucosa. Occasionally it
can involve extragenital site( rectal and pharyngeal gonorrhea). Initially
urogenital infection is symptomless and associated with purulent urethral
discharge, which responds to antibiotic therapy.Causative organisum is
niesseria gonorrhoea. It is an oval paired gram negative microorganisum.
Pathogenesis:
Once the gonococci are directly deposited on the genitourinary tract mucosa
during sexual intercourse, they penetrate through the intercellular spaces of
epithelium and reach the subepithelial connective tissue.
Within two to three days of infection an intense inflammatory reaction
occurs resulting in chracterstic mucopurulent discharge through urethral
lumen.
A chronic stage may be reached if untreated and spread is either by direct
extension through lyphatics and hematogenous route.
258
Clinical features
Disease of young adults between 15 to 24 years and is more common in
males. Incubation period of 1 to 2 weeks following contact with infected
persons.In females acute gonorrhea includes urethral cervical and vaginal
discharge.
Oral manifestations
Pharyngeal gonorrhea: Higher in pregnant women 2 to 15% and sexually
active homosexuals and heterosexuals practicing oral sex. Sore throat and
evidence of pharyngitis.
Gonococcal stomatitis: Incidence is very rare and often shows multiple
painful and round elevated gray white eroded spots with or without
pseudomembrane formation. Regional lymphadenopathy may be seen. Acute
gingivitis develops around extraction site in patients who practice fellatio
repeatedly for days after extensive dental extraction. Isolated ulcers,
gingivitis and membranous gingivostonatitis may develop.
Lips may develop acute painful ulcerations limiting the motion. Gingiva
may become erythmatous with or without necrosis. Tongue may present red
dry ulceration or become glazed and swollen with painful erosion with
similar lesion on buccal mucosa and palate.
259
COLOR PLATE-21
Gonococcal stomatitis (Gingival ulcerations, fiery red oral mucosa and yellowish
pseudomembrane)
260
Ulcer on tongue in
Gonococcal dermatitis syndrome
HEPATITIS
It is an acute, inflammatory and infective infection caused by hepatitis A, B,
C, D and NANB viruses. Dentists are 3 to 4 times more likely to be exposed
to hepatitis than with general population. Viral hepatitis may be type A, type
B, type non-A non-B which includes C,D,E.
ACUTE VIRAL HEPATITIS
In this most cases resolve completely within 4 months after onset of
symptoms but, some end in fulminating disease and others progress to
chronic hepatitis.
Clinical Features
Incubation period-Incubation period varies for type A-2 to 6 weeks, type B-1
to 6 months, non-A non-B 8-2 weeks to 6 months.
Symptoms: Hepatitis A has milder symptoms than other types of hepatitis.
Systemic complains include malaise, arthralgia, morbilliform skin rash,
261
anorexia, vomiting and myalgia. Patient has high grade fever with
tenderness and enlargement of liver. Patient also complains of upper
respiratory tract infection, distaste for cigarette, fever and enlargement of
liver. Arthritis and urticaria are common with hepatitis B and is probably due
to circulating immune complexes.
HEPATITIS A
It is a nonenveloped RNA virus (picarnovirus) transmitted via fecal-oral
route, by contaminated food and water. It is associated with poor personal
hygiene or overcrowded living condition.
Incubation period of 2 to 6 weeks. More common in children, primary and
nursery school age, it may occur as localized outbreaks or epidemic.
The period of infectivity is highest during the week before the onset of
clinical symptoms. Poor personal hygiene may be a cause in youngsters. In
262
265
266
PATHOGENESIS
Early in the HIV epidemic, it was recognized that this disease was caused by
a virus that gradually destroyed a hosts immune defenses, making virtually
all infected individuals susceptible to opportunistic infections. The particular
267
is
the
non-nucleoside
reverse
transcriptase
inhibitors
was
introducedprotease
inhibitors.
These
powerful
Fungal Infections
Candidiasis
Intraoral candidiasis, which is mainly caused by Candida albicans, is the
most common oral manifestation in patients with HIV disease. Although it is
not in itself pathognomonic for HIV disease, oral candidiasis may be an
indication of immune dete-rioration. A tentative diagnosis of oral candidiasis
is usually based on clinical appearance but should be confirmed by
laboratory tests. These tests include cytologic smears with potassium
hydroxide, biopsy for periodic acidSchiff and Gram staining for tissue
infiltration by spores and hyphae, or culture. In general, oral candidiasis has
four different clinical presentations, as follows:
1.Pseudomembranous candidiasis,or thrush
This condition is a common type of oral candidiasis. It manifests as white or
yellowish single or confluent plaques that can easily be rubbed off from the
oral mucosa. It is found on all oral surfaces and may leave an erythematous
or even bleeding underlying mucosa. Most patients are not aware of the
presence of this form
of candidiasis as pseudomembranous candidiasis is predominantly
asymptomatic. The condition is noticed most commonly when CD4 cells
counts drop below400 cells/mm3.
2.Erythematous or atrophic candidiasis
This condition appears on any mucosal surface as a reddish macular lesion,
atrophic patches, or depapillation on the dorsum of tongue . Erythematous
271
candidiasis
may
be
present
alone
or
in
combination
with
the
272
Pseudomembranous Candidiasis
Angular Cheilitis
273
Erythematous Candidiasis
Hyperplastic Candidiasis
nystatin troche (dissolved in the mouth four to five times per day).
Ointments and creams such as 1% clotrimazole ointment, 2% ketoconazole
cream, or nystatin cream are efficacious in treating angular cheilitis.
The efficacy of systemic antifungal medications may be significantly
reduced by impaired gastric acid secretion and by drug interactions with
medications such as rifampin. Furthermore, there exists a potential for liver
toxicity that needs to be addressed. Common systemic antifungals include
Ketoconazole(kenazole 200 mg tablets, one tablet twice a day with food),
fluconazole (flucot 100 mg tablets, 200 mg on day 1, followed by 100 mg
daily for 14 days), and itraconazole (itrole 100 mg tablets, 200 mg daily with
food). Resistance to fluconazole has been reported to occur in patients with
274
275
276
278
five times a day for 7 days). Prophylactic therapy with 800 mg acyclovir per
day may be necessary to prevent recurrence.
Varicella-Zoster Virus
There have been reports of increased incidence of human varicella-zoster
virus (HZV) infections among HIV-infected persons, relative to increased
age and degree of immunosuppression. Complications associated with HZV
in immunocompromised patients are common and can be severe, especially
for those individuals with CD4 counts fewer than 200 cells/mm3. 188
Clinically, oral HZV infection presents as vesicles that quickly rupture,
resulting in ulcerations. The ulcers are multiple, shallow, and small, with an
erythematous base, and are characteristically distributed unilaterally along a
division of the fifth cranial nerve. Patients frequently complain of pain,
neuropraxia, and tenderness. Although clinical presentation is distinct for
HZV infection, a definitive diagnosis should be confirmed by laboratory
tests such as histologic staining for multinucleated giant cells with
intranuclear inclusions, direct immunofluorescence, and cytology smears
taken from the lesion. Treatment usually is focused on supportive care and is
centered on the prevention of postherpetic neuralgia and dissemination.
High doses of oral acyclovir (800 mg orally five times a day), famciclovir
(virovir 500 mg orally three times a day), or valaciclovir (valcivir 500 mg
orally three times a day) have been efficacious in treating HZV infection.
Caution is needed when using valacyclovir in severely immunosuppressed
patients as this medication has been associated with hemolysis in this
particular patient population. For greatly immunosuppressed patients,
intravenous acyclovir therapy may be more appropriate. Foscarnet also may
be useful for acyclovir resistant herpes zoster.
Human Herpesvirus 8
279
diagnosis
should
also
include
bacillary
(epithelioid)
manifestations
with
papillomaviruses
are
similar
to
human
281
Kaposis Sarcoma
282
283
Color Plate-24
284
only affect the soft tissue, without any ulcerations, increased pocket depths,
or any attachment loss. Patients with this condition are usually
asymptomatic.Differential diagnosis should include a localized erythema
due to dry mucosa associated with mouth breathing, lichen planus,mucous
membrane pemphigoid, or an allergic reaction. The most recent theory
regarding the pathogenesis of this lesion implicates subgingival candida
infection as a possible cause.
Treatments include improved oral home care and conventional dental
scaling and root planing, along with the use of chlorhexidine gluconate
(0.12%) mouth rinses (15 mL swished and expectorated twice a day) for up
to 3 months. Additionally, concomitant use of topical antifungal medications
may be beneficial. Manifestations of NUG and NUP are triggered by
changes in the immune status, most probably aggravated by intraoral
bacteria. The two entities may present as a continuum of the same disease
but also may appear as separate entities. NUG is limited to the gingiva,
whereas NUP is characterized by localized to generalized aggressive
alveolar bone and attachment destruction. Occurrence of NUG has been
associated with stress, anxiety, malnutrition, and smoking. Patients with
NUG complain of spontaneous gingival bleeding and mild to moderate
gingival pain. NUP is associated with complaints of deep-seated bone pain,
spontaneous gingival bleeding, halitosis, and tooth mobility. Clinically, these
conditions are presented with initial lesions of limited crater like necrosis of
gingival papillae. When untreated, NUP may progress at a rate of 1 to 2 mm
of soft- and hard-tissue destruction per week. NUP is mostly seen with
severe immune suppression, with CD4 counts below 100 cells/mm3.A
definitive diagnosis is based on clinical evaluation and radiologic evaluation
285
Chlorohexidine
gluconate
(0.12%)
mouth
rinses
are
Nonspecific Ulcerations
Necrotizing Stomatitis: Necrotizing stomatitis is a localized acute painful
ulcerative lesion on mucosal surfaces overlying bone. This condition
eventually leads to necrosis of tissue and subsequent bone exposure. No
specific microbial agent or mechanism has been linked to its etiology. This
condition is seen in patients with CD4 cells fewer than 100 cells/mm3.168
Differential diagnosis includes aphthous ulcer and NUP.Treatment consists
of careful debridement, local or systemic steroid therapy, antibiotics, and
institution of a soft-tissue stent to protect the affected area from further
trauma and for delivery of topical medications.201
Aphthous Ulcers:
Recurrent aphthous ulcerations (RAUs) are idiopathic oral ulcerations.
There are three disease entities of RAUs: minor, major, and herpetiform.
Diagnosis of RAUs is a diagnosis of exclusion, the clinical impression
286
Treatment
for
major
aphthous
ulcerations
includes
patients.Certain
antiretroviral
therapies
induce
288
Administration of a growth factor such as granulocytemacrophage colonystimulating factor has shown to be successful in resolving ulcerations
associated with neutropenia.
Xerostomia
Xerostomia, or dry mouth, is a subjective symptomatic complaint that is
frequently noted by HIV-infected patients. It has been reported that reduced
salivary flow occurs in 2 to 10% of HIV-infected individuals.However, the
true prevalence is 80 to 90% of all patients taking HAART(Highly Active
Anti Retroviral Therapy). The effect of oral dryness on quality of life is
profound, and many patients with severe xerostomia sometimes opt to
change or stop their antiretroviral medications in order to regain better
salivary functions. The most common cause of decreased salivary flow in
HIVinfected patients is side effects of pharmocotherapeutic agents. Many
medications, such as antiretroviral medications (including nucleoside
transcriptase inhibitors, protease inhibitors) as well as antihistamines,
anticholinergics, antihypertensives, decongestants, narcotic analgesics, and
tricyclic antidepressants, have been associated with xerostomia. In addition,
xerostomia may be a result of HIV-associated salivary gland disease in this
population. The parotid glands are most frequently affected; however, minor
salivary glands can also be affected by viral infection such as with CMV.
Another cause of reduced salivary flow is radiation therapy to the head and
neck area, causing functional impairment of salivary glands in the radiated
area. Treatment for xerostomia focuses on symptomatic relief by
encouraging patients to hydrate themselves frequently and to minimize the
intake of caffeine and alcohol, which act as diuretics. Patients are also
recommended to use commercially available artificial saliva substitutes
289
are made to taste better by the addition of sugar formulations, which also
make them syrupy and sticky. It is advisable to have children rinse their
mouths with water after administration of these medications in order to
reduce the incidence of tooth decay.
GRANULOMA INGUINALE
It is also called as granuloma venereum, donovanosis. It is found in inguinal
and anogenital region and is caused by Donovan granulomatosis and is a
chronic slowly progressing, mildly contagious disease.
Clinical Features
It chiefly affects adult black of either sex, but may occur in any race. Lesion
begins as a small papule that ulcerates, increases in size and eventually gives
rise to velvety, beefy, granulating and spreadin ulcerative lesion of inguinal
and anogenital region. Pseudo-bubo-inguinal ulceration is commonly
secondary to the genital lesion and arises initially as fluctuant swelling
known as pseudo-bubo. Metastatic spread to bone and soft subcutaneous
tissue has been reported.
Oral Manifestations
Oral lesion appears to be the most common extra genital form of granuloma
inguinale. Oral lesions occur either as a result of autoinoculation through
infected fingers or through oral coitus. It is most commonly found on lips,
buccal mucosa or palate or they may diffusely involve the mucosal surface.
Lesions of lip are characterized by extensive superficial ulceration with well
defined elevated, granulomatous margins.
Types may be of three types, i.e. ulcerative, exuberant and cicatrical.
Ulcerative-It is painful sometimes bleeding issassociated with it.
291
LYMPHOGRANULOMA VENEREUM
It is a venereal disease caused by one of the three strains of Chlamydia
trachomatis.
Clinical Features
There may be fever, chills, headache and malaise. It persists as firm, tender
enlargement of inguinal lymph nodes. Nodes are tender and adherent to the
underlying tissues.Enlargement of lymph nodes both above and below the
inguinal ligament, is characcteristic feature called as 'groove sign'. Overlying
skin becomes reddened and dusky and multiple purulent fistulae develop
over enlarged glands, In females, placenta is frequently involved.
Marked scarring and local edema frequently develops secondary to
suppurative lymphadenitis.
Diagnosis is done by complement fixation test.
Oral Manifestations
Results due to orogenital contact. Tongue is the most common site.
292
Lesion consists of small, slightly painful superficial ulceration with nonindurated borders which appear on lip.In long-standing infection, there is
zone of cicatrical refraction, dark red area with loss of superficial
epithelium, or opaque lichenoid grayish papules.
Dysphagia, red soft palate and small red granulomatous lesions accompanied
by regional lymphadenopathy, are commonly associated symptoms. Cervical
lymphadenopathy is present. Skin covering the swollen nodes is violaaceous
and indurated usually with one or more draining sinuses.
MANGEMENT
Antibiotic therapy of tetracycline and erythromycin.
293
BIBLIOGRAPHY
REFERENCES
294
12. Wescott WB, Correll RW. Oral and perioral pigmented macules in a
patient with gastric and intestinal polyposis. J Am Dent Assoc
1984;108(3):3856.
13. Mignogna MD, Lo Muzio L, Ruocco V, Bucci E. Early diagnosisof
multiple hamartoma and neoplasia syndrome (Cowden syndrome): the role
of the dentist. Oral Surg Oral Med Oral Pathol Oral Radiol Endod
1995;79(3):2959.
14. Porter S, Cawson R, Scully C, Eveson J. Multiple hamartoma syndrome
presenting with oral lesions. Oral Surg Oral Med Oral Pathol Oral Radiol
Endod 1996;82(3):295301.
15. Katz JO,Chilvarquer LW,Terezhalmy GT. Gardners syndrome: report of
a case. J Oral Med 1987;42:2115. 3. Pearson TC.Apparent polycythemia.
Blood Rev 1991;5:20513.
16.3. Pearson TC.Apparent polycythemia. Blood Rev 1991;5:20513.17.4.
Cook JD, Skikne BS, Baynes RD. Iron deficiency: the global perspective.
Adv Exp Med Biol 1994;356:21928.
18.5. Provan D. Mechanisms and management of iron deficiency anemia. Br
J Haematol 1999;105 Suppl 1:1926.
19.7. Lindenbaum J.An approach to anemias. In: Bennett JC, Plum F,
editors. Cecil textbook of medicine. 20th ed. Philadelphia: WB Saunders
Co.; 1996. p. 823.
20.Shafer.W.G, Hine.M.K and Levy.B.M(1983) Dieases of blood and blood
forming
organs.In
textbook
of
oral
pathology(4the
719,W.B.Saunders,Philedelphia.
21. Greenberg MS. Clinical and histologic changes of the oral
mucosa in pernicious anemia. Oral Surg Oral Med Oral Pathol
1981;52:3842
295
edition)p-
296
297
syndrome
and
autoimmune
thyroiditis.
Nippon
Rinsho
1999;57:187881.
48. Baccarelli A. [Occupational agents and endocrine function:
updating of experimental and human data. Med Lav
1999;90:65070.
49. Dunn JT, Dunn AD. The importance of thyroglobulin structure
for thyroid hormone biosynthesis. Biochimie 1999;
81:5059.
50. Udelsman R, Chen H. The current management of thyroid
cancer. Adv Surgery 1999;33:127.
51. Fadda G, LiVolsi VA. Histology and aspiration cytology of
benign thyroid diseases. Rays 1999;24:18296.
52. Meller J, Becker W. Scintigraphy with 99mTc-pertechnetate
in the evaluation of functional thyroidal autonomy. Q J Nucl
Med 1999;43:17987.
53. Pacini F, Pinchera A. Serum and tissue thyroglobulin measurement:
clinical applications in thyroid disease. Biochimie
1999;81:4637.
54. Walsh RM,Watkinson JC, Franklyn J. The management of the
298
301
88.
Callen
JP.
Collagen
vascular
diseases.
Med
Clin
North
Am1998;82:121737.
89. Jimenez SA, Hitraya E, Varga J. Pathogenesis of scleroderma: collagen.
Rheum Dis Clin North Am 1996;22:64774.
90. Silver RM,Heyes MP,Maize JC, et al. Scleroderma, fasciitis and
eosinophilia associated with the ingestion of tryptophan. N
Engl J Med 1990;322:874-81.
91. Steen VD. Clinical manifestations of systemic sclerosis. Semin Cutan
Med Surg 1998;17:4854.
92. Steen VD,Conte C,Owens GR, et al. Severe restrictive lung disease in
systemic sclerosis. Arthritis Rheum 1994;37:12839.
93. Rademaker M,Meyric K, Thomas RH, et al. The anti-platelet effect of
nifedipine in patients with systemic sclerosis. Clin Exp Rheumatol
1992;10:57.
94. Rook AH, Freundlich B, Jegasothy BV, et al. Treatment of systemic
sclerosis with extracorporeal photochemotherapy: results of a multicenter
trial.Arch Dermatol 1992;128:337-46.
95. Nagy G, Kovacs J, Zeher M, et al. Analysis of the oral manifestations of
systemic sclerosis. Oral Surg Oral Med Oral Pathol 1994;77:1416.
96. Montesi A, Pesaresi A, Cavalli ML, et al. Oropharyngeal and esophageal
function in scleroderma.Dysphagia 1991;6:219-23.63101. Callen JP. Oral
manifestations of collagen vascular disease. Semin Cutan Med Surg
1997;16:3237.
97. Rubin MM, San Filippo RJ. Resorption of the mandibular angle in
progressive systemic sclerosis: case report. J Oral Maxillofac Surg
1992;50:75-7.
302
303
immunodeficiency
virus:
clinical
presentations,diagnosis,
305