The physician made the presumptive diagnosis of sarcoidosis. The involvement of the rim of the nasal ala is especially common in cutaneous sarcoidosis.
Sarcoidosis is a granulomatous disease that involves multiple organ systems; the etiology is unknown. Cutaneous manifestations occur in about 25% of systemic sarcoidosis patients.
This patient had lupus pernio in which the sarcoid granulomas involve the face (as one might see in lupus erythematosus). Other less common types of cutaneous sarcoidosis can be maculopapular, nodular, or infiltrative within existing scars or tattoos. Erythema nodosum is a common nonspecific finding in sarcoidosis. Other less common nonspecific lesions reported in sarcoidosis include erythema multiforme, calcinosis cutis, and lymphedema. Nail changes can include clubbing, onycholysis, subungual keratosis, and dystrophy.
In this case, the diagnosis was confirmed with a punch biopsy of a lesion on the upper lip (above the vermillion border), as a biopsy of the nasal ala is more likely to leave undesirable scarring. Chest radiographic involvement is seen in almost 90% of patients and is used to stage the disease.
Stage I disease shows bilateral hilar lymphadenopathy (BHL). Stage II disease shows BHL plus pulmonary infiltrates. Stage III disease shows pulmonary infiltrates without BHL. Stage IV disease shows pulmonary fibrosis. This patient had stage I disease.
Cutaneous involvement of sarcoidosis is typically not life threatening; treatment is aimed at minimizing disfigurement. In this case, the lesions were painful and treatment involved a mid-potency topical steroid.
Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Sarabi K, Khachemoune A. Sarcoidosis. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. The Color Atlas of Family Medicine. New York, NY: McGraw-Hill; 2009:740-744.
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