Subcutaneous Sarcoidosis

For the past 3 months, a 47-year-old woman suffered from progressive malaise, fatigue, and exertional dyspnea. Small, nontender plaques first developed over her knees, and she now had symmetric, claylike plaques over her forearms and hands. These patches extended from the olecranon along the ulnar side of her forearm, wrapped around her wrist, and extended across the dorsum of her hand to the middle finger.

Results of laboratory studies were normal except for significant hilar adenopathy on the chest film, a modest restrictive defect on pulmonary function tests, an erythrocyte sedimentation rate of 43 mm/h, and an elevated angiotensin-converting enzyme level. Incisional biopsy revealed a dense, granulomatous infiltrate involving the lower dermis and spilling over into the subcutis.

The histologic findings confirmed the diagnosis of subcutaneous sarcoidosis. Once the patient started to take oral prednisone, 40 mg/d, she experienced dramatic improvement in energy levels and overall sense of well-being. The cutaneous signs resolved completely in 6 weeks.

Subcutaneous sarcoidosis typically presents on the legs as nontender, erythematous nodules that resemble erythema nodosum. The latter condition may be associated with sarcoidosis, particularly as part of Lfgren's syndrome (sarcoidosis with erythema nodosum, hilar adenopathy, arthritis, synovitis, and/or uveitis).

Drs Jeffrey J. Meffert and Dirk M. Elston of Fort Sam Houston, Tex, note the importance of distinguishing erythema nodosum associated with sarcoidosis from the far rarer pure subcutaneous sarcoidosis; Lfgren's syndrome usually has a very good prognosis, whereas subcutaneous sarcoidosis is almost universally associated with systemic disease. Punch biopsy may not yield adequate tissue, and incisional biopsy through the full thickness of the dermis is usually required.