BACKGROUND: Sarcoidosis is a chronic granulomatous multisystem disease of unknown origin. Cutaneous manifestations are common but ulcerative skin lesions rarely occur. We report a case of generalized ulcerative sarcoidosis in a patient with fatal multisystem involvement. A review of the literature revealed only 34 other cases of ulcerative sarcoidosis, 5 of which were generalized. We reviewed the clinicopathologic spectrum of the disease and available treatment options. OBSERVATIONS: Ulcerative sarcoidosis affects women (23 of 35 cases) and blacks (18 of 30 cases) most commonly and occurs in young adulthood. Cutaneous sarcoid lesions, including ulcers (11 of 26 cases), were the presenting complaint in 26 of 35 cases. Ulcers developed both de novo and within other sarcoid lesions. The lower extremities were most often involved (29 of 35 cases). A histological diagnosis was made using skin biopsy specimens in all cases. Corticosteroid therapy seems most effective for treatment of ulcerative sarcoidosis, with methotrexate a proven alternative for refractory cases. CONCLUSIONS: Dermatologists most often make the initial diagnosis of sarcoidosis in patients with ulcerative lesions. In the proper clinical setting, a skin biopsy of ulcerative lesions establishes the diagnosis and prompt treatment reduces morbidity.
BACKGROUND:Sarcoidosis is a chronic granulomatous multisystem disease of unknown origin. Cutaneous manifestations are common but ulcerative skin lesions rarely occur. We report a case of generalized ulcerative sarcoidosis in a patient with fatal multisystem involvement. A review of the literature revealed only 34 other cases of ulcerative sarcoidosis, 5 of which were generalized. We reviewed the clinicopathologic spectrum of the disease and available treatment options. OBSERVATIONS: Ulcerative sarcoidosis affects women (23 of 35 cases) and blacks (18 of 30 cases) most commonly and occurs in young adulthood. Cutaneous sarcoid lesions, including ulcers (11 of 26 cases), were the presenting complaint in 26 of 35 cases. Ulcers developed both de novo and within other sarcoid lesions. The lower extremities were most often involved (29 of 35 cases). A histological diagnosis was made using skin biopsy specimens in all cases. Corticosteroid therapy seems most effective for treatment of ulcerative sarcoidosis, with methotrexate a proven alternative for refractory cases. CONCLUSIONS: Dermatologists most often make the initial diagnosis of sarcoidosis in patients with ulcerative lesions. In the proper clinical setting, a skin biopsy of ulcerative lesions establishes the diagnosis and prompt treatment reduces morbidity.