Bilateral condylar resorption in dermatomyositis

A case report

Michael T. Brennan, DDS,a Nicholas J. Patronas, MD,b and Jaime S. Brahim, DDS, MS,c
Bethesda, Md
NATIONAL INSTITUTES OF HEALTH

Polymyositis is an inflammatory disease commonly affecting the striated muscle. When it is accompanied by charac-
teristic skin lesions, the condition is called dermatomyositis. Bilateral condylar resorption has been reported with autoimmune
conditions and chronic systemic steroids. We report the first documented case of bilateral condylar resorption in a patient with
dermatomyositis. Possible etiologic factors and treatment outcomes are discussed. (Oral Surg Oral Med Oral Pathol Oral
Radiol Endod 1999;87:446-51)

Polymyositis (PM) and dermatomyositis (DM) are a

group of inflammatory diseases primarily affecting
skeletal muscle. The most common clinical manifesta-
tion is proximal muscle weakness. These inflammatory
myopathies are characterized by increased muscle
enzymes (most commonly, creatine kinase), abnormal
electromyographic findings, and distinct histopatho-
logic findings. Cutaneous manifestations of DM distin-
guish it from PM. Women are more commonly affected
than men, with peak incidences during childhood and
between the ages of 45 and 65 years. The incidence is
1 to 10 cases per million people each year.1 DM and
PM have been further divided into 5 categories, as
follows: (1) idiopathic PM, (2) idiopathic DM, (3)
DM/PM associated with malignancy, (4) childhood
DM/PM, and (5) DM/PM associated with another
connective tissue disease.2
We present an unusual case of bilateral condylar
resorption in a patient with idiopathic DM and review
the literature. This head and neck manifestation has not
previously been reported in the DM/PM literature. Fig 1. Presurgical profile shows skeletal deformity.

CASE REPORT
A 37-year-old man was referred to the National Institute of
the late 1970s, he had been diagnosed as having dermatitis
Dental Research Dental Clinic by his rheumatologist with a
herpetiformes. In 1983, he had complained of arthralgias,
complaint of bilateral jaw pain and malocclusion. The initial
malaise, and profound muscle weakness; findings of a muscle
symptoms of temporomandibular joint (TMJ) problems had
biopsy at that time were consistent with DM. The patient also
begun in February 1986; at that time, the patient described his
had antinuclear antibodies to the Jo-1 antigen. His course has
teeth as “not meet[ing] the same way that they did before.” In
been complicated by several chronic infections, including
pulmonary Mycobacterium avium intracellulare, Salmonella,
aFellow (Oral Medicine), Gene Therapy and Therapeutics Branch, and subacute bacterial endocarditis secondary to bicuspid
National Institute of Dental Research. aortic valve. The patient has a history of skin ulcerations with
bDiagnostic Radiology Department, Chief, Neuroradiology Section,
slow healing, laryngeal abscess, and laryngeal papilloma.
Clinical Center.
cSenior Staff, Oral and Maxillofacial Surgery, Clinical Research
At the time he was admitted, the patient was taking the
Core, National Institute of Dental Research.
following medications: prednisone, 30 mg, alternating with 5
Received for publication June 15, 1998; returned for revision July mg every other day; dapsone, 75 mg, alternating with 100 mg
13, 1998; accepted for publication Dec 1, 1998. every other day; chlorambucil, 6 mg every day; iron, 1 tablet
Copyright © 1999 by Mosby, Inc. every day; calcium carbonate, 650 mg every day; and multivi-
1079-2104/99/$8.00 + 0 7/13/96543 tamin, 1 every day. The patient has recently been treated with

446
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Brennan, Patronas, and Brahim 447
Volume 87, Number 4

Fig 2. Presurgical panoramic radiograph shows bilateral condylar resorption.

methotrexate every week and high-dose prednisone to bring

his myositis under control. He has done fairly well on this
regimen.
On examination. the patient’s profile was suggestive of a
skeletal class II pattern (Fig 1). The right and left TMJs were
not tender, and no deviation on mandibular opening was
observed. Lateral movement of the mandible was normal. An
open bite of 4 mm at the midline and a 2-mm opening at the
right and left canines was present. Radiographic evaluation
with polytomography of the TMJs and a panoramic radi-
ograph (Fig 2) showed erosions and deformities of the
mandibular condyles.
The patient was given an appointment to return to the clinic
in 6 months for a follow-up. He was followed for approxi-
mately 21⁄2 years. His open bite continued to increase, eventu- Fig 3. Presurgical occlusion shows open bite.
ally reaching 8 mm at the midline (Fig 3). A computed tomo-
graphic (CT) scan performed at that time revealed complete
resorption of the mandibular condyles and marked erosions of rior segment, approximately 4 mm of anterior intrusion, and a
the mandibular fossae, the left being more severely affected repositioning of 4.5 mm posteriorly of the anterior segment.
than the right (Fig 4). The segments were secured in place with plates and screws.
During the 21⁄2 years of close follow-up after initial presen- The patient tolerated the surgical procedure well and has been
tation, the patient’s main complaints were his inability to followed for 7 years without relapse.
close his lips and bring his teeth into contact and the difficulty An examination performed 7 years postoperatively revealed
he had when eating. When no changes in occlusion were a satisfactory range of motion, with a 49-mm opening from
noted for a 6-month period, surgical treatment was planned. incisal edge to incisal edge, 7 mm of right lateral motion, and
Because of the skeletal class II malocclusion, lip incompe- 10 mm of left lateral motion. An occlusal comparison showed
tence, lack of TMJ pain, and good function, a segmental Le stability of the occlusion 7 years after the surgery (Figs 5 and
Fort I osteotomy was recommended instead of an unpre- 6). A CT and a panoramic radiograph obtained at the 7-year
dictable TMJ surgery for the correction of his deformity. An postoperative follow-up showed the mandibular condyles to
orthodontic consultation was obtained to prepare the patient be larger in size but fragmented. The overall increase in the
for the surgical correction of his facial deformity through a size of the condyles suggested remodeling (Fig 7).
segmental Le Fort I osteotomy. The patient went through 1
year of orthodontic treatment to prepare his occlusion.
DISCUSSION
As a result of an infectious disease consultation regarding
The primary clinical manifestation of DM/PM involves
preoperative antibiotic therapy, prophylaxis with 2 grams IV of
ampicillin and 125 mg of gentamycin for subacute bacterial muscular disease. Proximal muscle weakness is the most
endocarditis prophylaxis were recommended. A segmental Le common presentation. In addition, dysphagia and
Fort I osteotomy was performed through use of a standard dysphonia may result from involvement of the
technique; there were no complications, and the patient was esophageal, pharyngeal, and flexor muscles of the neck.3
placed in intermaxillary fixation. A stable occlusion was Respiratory compromise may result from involvement of
obtained, with approximately 7 mm of intrusion of the poste- the respiratory muscles.
448 Brennan, Patronas, and Brahim
Fig 4. Presurgical CT scan shows bilateral resorption of condyles.
Fig 5. Final occlusion after treatment.
Cutaneous involvement in DM includes a violaceous
discoloration affecting the eyelids, bridge of nose, and
cheeks. This characteristic finding, known as a
heliotrope rash, is found in 60% of patients with DM.1
Seventy percent of patients with DM will develop
Gottron’s papules, which are erythematous to viola-
ceous papules on the knuckles, elbows, and knees.1 An
erythematous skin rash may also affect the neck, upper
arms, and trunk. Skin calcifications—calcinosis cutis—
may occur because of calcium carbonate nodules.4
The presence of arthralgias has been reported in 20%
to 50% of cases.5 Citera et al5 evaluated 29 patients with
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
April 1999
Fig 6. Occlusion at 7-year follow-up.
DM/PM (excluding DM associated with another connec-
tive tissue disease) and found 27.5% to have symptoms
of arthritis. Additionally, the arthritis group showed a
significant higher frequency for men.5 Other manifesta-
tions include a multitude of pulmonary, cardiac, gastroin-
testinal, and ophthalmologic manifestations.
Cunningham and Lowry6 reported that 55% of
patients with DM/PM had clinical signs or symptoms
within the head and neck region. As previously
mentioned, a heliotrope rash and dysphagia resulting
from myopathy may occur in patients with DM. In addi-
tion, facial swelling of the periorbital region and lips
may be present. A “generalized stomatitis,” including
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
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Fig 7. CT scan at 7-year follow-up shows larger condyles, suggestive of remodeling.
gingivitis and glossitis, has been observed in one third of
patients with DM.7 Lesions similar in appearance to
those seen in aphthous stomatitis, erosive areas of the
lips and palate, and erythema of the buccal mucosa with
telangectasias have been reported.7 A delay in exfolia-
tion of the deciduous teeth because of a lack of root
resorption and soft tissue calcifications has been found
in children with DM.8
Carcinoma is found in 15% to 25% of patients with
DM/PM, this incidence being 5 to 11 times that in the
general population.1 In addition, a patient with DM has
a 20% greater risk of developing a neoplasia than does
a patient with PM.3 Many types of malignancies have
been associated with DM/PM. The most prevalent
types of neoplasms involve the breast, lung, ovary,
stomach, and uterus.6
The mainstay of treatment for DM/PM is cortico-
steroids; they are the still the empiric drugs of choice,
though no double-blind studies have been performed.
A true double-blind clinical trial comparing cortico-
steroids with no treatment would probably have dire
consequences because of the poor prognosis associated
with untreated myositis.9 Corticosteroids are usually
given in a dose of 1 to 2 mg/kg/day. Approximately
20% of patients will not respond adequately to corti-
costeroids and will require other medications.10
Condylar resorption has not previously been reported
with DM/PM. Possible causes for the condylar resorp-
tion seen in our patient include disease manifestation
of DM, steroid-induced resorption, mechanical stress,
and a combination of these factors.
Brennan, Patronas, and Brahim 449
Condylar resorption has been associated with other
connective tissue diseases. The incidence of mandibular
resorption in systemic sclerosis ranges from 20% to
33%, with the resorption affecting the condyle in 20.8%
of these cases.11 The resorptive pattern in systemic scle-
rosis is localized around muscular insertions and is
thought to result from pressure ischemia and vascular
ischemia.11 The incidence of TMJ involvement for
rheumatoid arthritis (RA) is thought to be greater than
50% and is considered a later disease manifestation.
Numerous cases of bilateral condylar resorption in RA
have been reported in the literature.12 As noted previ-
ously, a 27.5% prevalence of arthritic involvement has
been established in patients with DM/PM. In these
patients, the most commonly affected joints were the
knees and proximal interphalangeal joints; involvement
less commonly involved the metacarpophalangeal joints,
wrists, and elbows. No TMJ involvement was noted.
Ogden,12 reviewing 12 RA cases with resorption of
the condyle, noted that 9 of the 12 patients were taking
at least 5 mg of prednisolone per day for 4 years. Further
analysis of these patients with RA who were treated with
systemic steroids showed no radiographic changes after
2 years of steroid therapy; however, gross resorption
after 5 years of systemic steroid therapy was noted.12
In the present case, the possible causes of the
condylar resorption include steroid-induced bone
resorption. The possibility that steroids are an etiologic
factor in condylar resorption has been considered in
cases reported with RA,12 myasthenia gravis,13 and
treatment of renal transplant rejection.14 Evidence of
450 Brennan, Patronas, and Brahim
joint changes resulting from corticosteroids also is seen
in the femoral head. Corticosteroids have been associ-
ated with avascular necrosis of the femoral head in 12%
to 30% of renal transplant patients receiving cortico-
steroids15 and in 2.8% to 16% of patients with systemic
lupus erythematous receiving corticosteroids.16 Acton14
noted radiographic similarities between aseptic necrosis
of the femoral head and resorption of the condyle.14
In vivo and in vitro studies have demonstrated adverse
effects of corticosteroids on metabolism and growth of
the mandibular condyle. Receptors for triamcinolone
were present on the condylar cartilage cells of neonatal
mice; receptor binding of this steroid was shown to
inhibit DNA synthesis in the mice.17 Additional studies
analyzing the effect of corticosteroids on the condyles of
neonatal mice have shown numerous metabolic effects:
inhibition of proteoglycan synthesis,18 suppression of
collagen synthesis,19 inhibition of RNA and protein
synthesis,20 and stimulation of matrix vesicles before
mineralization of cartilage.21 Silbermann and Maor20
examined the effects of intraarticular injections of
glucocorticoids in baboon TMJs. The local effect of the
steroids included chondrolysis and osteolysis. A
systemic effect of corticosteroids was also noted with
signs of hyperparathyroidism, including hypercalcemia
and hypophosphatemia. More evidence of a secondary
hyperparathyroid state affecting TMJ resorption was
reported in hemodialysis patients.22 Condylar resorption
was noted in 6 of 39 patients receiving prolonged
hemodialysis for renal failure. Such resorption was
attributed to secondary hyperparathyroidism resulting
from hemodialysis. This points to the importance of the
fact that corticosteroids have local and systemic mecha-
nisms of condylar resorption.
A normal adaptive response results in equal anabolic
and catabolic biologic responses to TMJ functional
demands. Disruption of a biologic response may lead
to a cascade of events resulting in joint degeneration.
In the present case, biomechanical stresses also should
be considered an etiologic factor of condylar resorp-
tion. Milam and Schmitz23 proposed 3 mechanisms of
injury associated with biomechanical stresses: direct
mechanical injury, neurogenic inflammation, and
hypoxia-reperfusion. Direct mechanical injury can lead
to such biologic changes as production of tissue-
damaging free radicals and disruption of nutrient supply.
Neurogenic inflammation is characterized by release of
neuropeptides from activated peripheral nerves; the
release of these neuropeptides may enhance inflamma-
tory responses. Hypoxia-reperfusion may lead to tissue
hypoxia from the disruption of blood flow resulting from
higher intracapsular hydrostatic pressure.
Specific biologic markers have been associated with
early cartilage degradation of the TMJ. Kubota et al24
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
April 1999
noted the presence of IL-1β, IL-6, matrix metallopro-
teinase 2, and matrix metalloproteinase 9 in TMJ
synovial fluid. In addition, proteoglycan components
(eg, keratin sulfate) were isolated in synovial fluid
from joints with early osteoarthritis.25
The condylar resorption in the present case is probably
caused by a combination of disease manifestation,
steroid-induced resorption, and/or mechanical stress.
When it is considered that no reports of disease-associ-
ated condylar resorption with DM/PM have been
reported in the literature, direct disease manifestation
without contributing factors seems unlikely. That steroid
therapy is the only contributing factor in condylar resorp-
tion is also unlikely because the common use of systemic
corticosteroids in the treatment of numerous diseases
does not result in universal bony resorption. The primary
treatment of DM/PM is systemic corticosteroids, by
means of which the condition is appropriately controlled
in 80% of patients.1 The absence in the literature of
condylar resorption in DM/PM cases treated primarily
with corticosteroids indicates that in the present case
there were probably additional factors leading to the
condylar resorption. No clinical symptoms of pain,
clenching, or bruxism were noted by our patient. In addi-
tion, no history of trauma was reported. The only symp-
toms or signs of TMJ dysfunction were the patient’s
increasing anterior open bite, his chief complaint of diffi-
culty in eating, and his inability to put his lips and teeth
together. Strong evidence for mechanical stress–induced
resorption is not supported by clinical signs and symp-
toms. Subclinical biomechanical stress to the condyles
cannot be discounted. Accordingly, although the exact
cause of condylar resorption cannot be specified in the
case of our patient, factors related to biologic effects of
corticosteroids, pathologic changes related to DM, and
unspecified biomechanical stress were likely contributing
factors in the observed degenerative process.
Follow-up findings in the present case indicate that 7
years after orthognathic surgery, the patient has main-
tained his occlusion without signs of skeletal and/or
occlusal relapse. CT scanning of the mandible showed
larger functioning condyles, which suggests remod-
eling. The results indicate that in this patient the treat-
ment of the open bite resulting from bilateral condylar
resorption with a Le Fort I osteotomy was more appro-
priate than TMJ surgery would have been.
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Reprint requests:
Jaime S. Brahim, DDS, MS
NIDR, NIH
Bldg. 10 Room 1N-113
10 Center Drive MSC 1190
Bethesda, MD 20892