Dr.

Eisenberg
January 17, 2014

Oral Manifestations of Systemic Diseases I

Systemic Process
Any influence or condition with effects that are reflected widely throughout the body

Systemic Influences
Genetic
Hormonal
Metabolic
Immunologic
Hematologic
Neurologic
Infectious
Neoplastic
Idiopathic (Uncertain)

PRINCIPLES
History
Clinical Findings
Radiographic Fidings
Laboratory/Other Test Results

Oral Findings Suggestive of Systemic Disease:

History
(+) Hx of dxd systemic influence or condition
Oral lesions onset concomitant with onset of extraoral findings
Oral problem regresses/resolves with regression of extraoral manifestations
Oral complaint(s) in temporal relationship with flare of systemic problem
Oral Findings Suggestive of Systemic Condition: Clinical
Lesions widely distributed
Broad spectrum of lesions (eg. blisters, ulcers, others)
Constitutional symptoms, signs
Resolution of oral lesions with tx, elimination of systemic condition
Oral lesions recur in absence of local cause
Findings Suggestive of Systemic Disease:
Teeth & Jaws
Radiographs: widespread changes in bone architecture
Asymmetry of jaw(s)
Unexplained tooth mobility
Pain, symptoms unexplained by local factors
Unexplained changes in bite and/or tooth loss in adult; abnormal 1 tooth loss
Findings Supportive of Systemic Cause:
Special Studies
Blood, urine studies (CBC, electrolytes, enzymes, microbial cultures, etc.)
Immunological testing (serological,skin hypersensitivity, etc.)
Radiographs, special imaging
Cytology (smears, aspirates)
Tissue biopsies
Selected Osseous Manifestations of Systemic Influence
Hyperparathyroidism
Pagets Disease
Granulomatous Diseases sarcoidoisis, Crohns, cheilitis granulomatosa

1 Hyperparathyroidism (HPT)
F>>M, > 60 years old
8090% Parathyroid adenoma
1015% Parathyroid hyperplasia
< 2% Parathyroid malignancy
PTH secretion serum, urine Ca, PO4
Stones, bones, abdominal groans
Mental disturbances
2 Hyperparathyroidism

Bone Lesions, HPT

Subperiosteal resorption, phalanges
Loss of lamina dura (jaws)
Giant cell lesions (Brown tumors): solitary or multiple
Osteitis fibrosa cystica (von Recklinghausens disease of bone)
Pathologic fxs
Renal osteodystrophy: groundglass F.D.like changes
Pagets Disease of Bone
Unregulated bone resorption and apposition weakened osseous structures
EtiologyInflammatory, genetic, infectiousslow virus?
Mutations in Sequestome 1 gene
(SQSTM 1)regulates osteoclastic activation
(Rare instances of Pagets as component of a syndrome with myopathy, dementia, etc.)
Some cases are familial; some are sporadic
Pagets Disease of Bone
Serum alkaline phosphatase levels are significantly elevated

Poorly regulated bone apposition and resorption produces a histologically mosaic pattern

Nonphysiologic resorption and apposition of bone throughout skeleton. Lytic and cotton woollike osseous
changes on radiographs.
Note highly irregular (mosaic) pattern. Bone is hypervascular.
Granulomatous Diseases
Sarcoidosis
Crohns Disease
Cheilitis Granulomatosa/ Orofacial Granulomatosis

Sarcoidosis
Etiology uncertain
Hypersensitivity to atypical mycobacteria?
Related to M. Tuberulosis (DNA/RNA in affected tissue)?
Susceptibility dependent on HLA ?
Cellmediated immune dysregulation
(Anergy, circulating Tlymphocytes, lymphocyte transformation)
Symptoms: fatigue, lethargy, appetite
Organspecific complaints:
Cutaneous: lupus pernio, erythema nodosum
Pulmonary: cor pulmonale, pulmonary HTN
Lymphadenopathy
Ocular: uveitis, uveoparotid fever
Hepatic
Clinical Features
2nd 4th decades
Females > Males
Blacks > Whites
Variable course: insidious onset, spontaneous remissions, chronic progression possible
Laboratory Features
Noncaseating granulomas
 Serum Ca ++; ACE1
Chest rads, jaw rads
Kveim test(+)\\

Sarcoidosis: Possible Oral Findings*

Soft tissue nodules
Salivary gland enlargement
Destruction of alveolar bone (diffuse)
Tooth mobility
* Noncaseating granulomatous inflammation

Crohns Disease*
Chronic granulomatous, panG.I. disease
Immunologicallymediated
Onset often in teens
 Abdominal cramping, diarrhea, hematochezia
Anemia, fatigue
Oral lesions do not necessarily correlate with intestinal exacerbations
* Crohns granulomatosis, Granulomatous ileitis
Potential Oral Lesions in Crohns Disease
Aphthoustype ulcerations
Fissures
Cobblestonelike hyperplastic soft tissue masses, especially in the vestibules
Pyostomatitis vegetans
Swellings of the lips (granulomatous cheilitis)
Langerhans Cell Disease
(Formerly Histiocytosis X)
LettererSiwe Disease*
HandSchullerChristian Disease
Eosinophilic Granuloma
*LS D: Clinical/laboratory features more c/w malignant lymphoma, not LCD

Langerhans Cell Disease

Systemic disease affects multiple organ systems including bone
Proliferation of dendritic mononuclear cells c/w Langerhans cells
Monoclonal proliferation of lesional cells
c/w a neoplastic process
Systemic condition
Proliferation of (histiocytelike) Langerhans cells in bone, soft tissue
Male predilection
~50% of cases in children < 10 yrs of age
Bone lesions may be solitary, multiple, multifocal
Skull, ribs, long bones, jaws

Langerhans CellsDx Features

Immunoreactivity for:
CD207
CD1a
S100
E.M.
Birbecks granules
Langerhans Cell Disease
Clinical and Radiographic features
> 50% in young children
Males = females
Skeletal involvement widespread or isolated to one bone
Skull, ribs, vertebrae, mandible commonest sites
Round or ovoid, uncorticated radiolucencies mimics severe perio bone loss
Ulcerative mucosal, gingival and cutaneous lesions

Langerhans Cell Disease

Bone Involvement
Skull, femur (< 10 yrs of age);ribs, shoulder girdle, mandible (> age 10)
Jaws involved in 1020% of cases
Tenderness, dull pain
Alveolar bone loss (resembles severe periodontitis radiographically)
Periapical radiolucencies (well demarcated, noncorticated)
Tooth mobility
Treatment, LCD
Accessible bone lesions curettage
Localized bone lesions: intralesional steroid injections
Rads tx: may be contraindicated for children
Chronic, disseminated disease:
Chemotherapy: combined agents including prednisone/vincristine
Regular clinical follow up