Clinical History:
26 year-old female with soft tissue swelling, joint pain and stiffness in
the right hand.
Figure 1
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Radiographic Findings:
Frontal radiograph of the right hand shows a diffuse lacy reticular lytic
process involving the proximal and middle phalanges of the fourth digit
as well as in the proximal phalanges of the first and second digits. There
is some periosteal thickening in the fourth digit proximal phalanx. Soft
tissue swelling is also present in the involved digits.
Discussion:
Sarcoidosis is a systemic idiopathic granulomatous disorder that affects
men and women almost equally, usually between 20-40 years of age. This
diagnosis is established by demonstration of noncaseating granulomas in
tissue and exclusion of other causes of granulomas. There is a racial
predisposition in which blacks are affected more than whites in the U.S.
by approximately 10:1.
This disease is characterized by abnormal hilar and mediastinal lymphadenopathy,
pulmonary infiltrates, skin lesions in which erythema nodosum is the most
common cutaneous manifestation and ocular abnormalities in which the uveal
tract is most commonly involved. Central nervous system abnormalities,
not limited to meningitis or encephalopathy, are rarely seen. Commonly,
patients with sarcoidosis are asymptomatic and present with an abnormal
chest radiograph obtained for other reasons. Less than 5% of patients
who have sarcoidosis have a normal chest radiograph. A favorable prognosis
has been reported in patients with erythema nodosum, hilar adenopathy,
no extrapulmonary involvement and normal to near-normal pulmonary function
studies. Mortality approaches 5-10% and is usually related to respiratory
failure or cor pulmonale.
Laboratory findings include: anemia, leukopenia, eosinophilia, low albumin,
high globulin, hypercalcemia, hypercalciuria, elevated serum phosphate,
lymphocytopenia, cutaneous anergy, elevated angiotensin converting enzyme
levels and pituitary dysfunction which may cause patients to present with
diabetes insipidus.
Osseous involvement occurs in approximately 5% of patients and most commonly
affects the bones of the hands and feet. Multiorgan disease is usually
present when osseous involvement occurs. These findings include:
- Asymmetric small lytic appearing lesions which can form a honeycombed,
lacy appearance. Periosteal reaction is rare.
- Involvement of the middle and distal phalanges seen more commonly
than the proximal phalanges.
- Cystic lesions with or without endosteal scalloping, which may show
a thin rim of sclerosis associated with lesions as they heal.
- Acro-osteosclerosis, telescoping, bony fragmentation and pathologic
fracture.
- Osteopenia.
- Sclerotic bone lesions, typically seen in the axial skeletal structures.
- Nodular soft tissue swelling secondary to noncaseating granulomas
present in the skeletal muscle.
- The joints are uncommonly involved, if present there is typically
peripheral and symmetrical involvement of the small and medium sized
joints such as the ankles, knees, and wrists. An acute arthritic form
is described with swelling and pain which is typically out of proportion
to the radiographic signs of inflammation. A chronic form is also described
which can progress to osteoarthritis or erosive osteoarthritis.
The standard therapy for symptomatic sarcoidosis is administration of glucocorticoids.
However there is no definite proof that such therapy influences long-term
outcome.
Diagnosis:
Musculoskeletal findings of sarcoidosis affecting the hand
References:
- Resnick D, Niwayama G. Sarcoidosis. In: Diagnosis of
Bone and Joint Disorders. 2nd ed. Philadelphia, Pa: W.B. Saunders, 1988;
4012-4030.
- Staton G, Ingram R. Sarcoidosis. In: Scientific American
Medicine. Scientific American Inc. New York, New York: 1978-99; 8-12.
- Lawson JP. The Joint Manifestations of the Connective
Tissue Diseases. In: Seminars in Roentgenology. Philadelphia, Pa: W.B.
Saunders, 1982; 25-38.
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