Sarcoidosis: Classic Mahogany Papules and Lupus Pernio Associated With Chronic Fibrotic Sarcoidosis

January 2, 2009

These firm papules, a classic sign of sarcoidosis, are seen around the eyes of a 27-year-old woman (A) and the external ear of a 33-year-old woman (B).

Classic Mahogany PapulesLupus Pernio Associated With Chronic Fibrotic SarcoidosisPruritic Annular Lesions

Classic Mahogany Papules

These firm papules, a classic sign of sarcoidosis, are seen around the eyes of a 27-year-old woman (A) and the external ear of a 33-year-old woman (B).

 

 

Other cutaneous manifestations include hypopigmented patches commonly overlying subcutaneous masses, redness and scaling of the lower legs, erythematous papules on the eyelid margins or the nares, and bone cysts in the fingers that produce visible swelling.

 

 

(Case and photographs courtesy of Dr Mary C. Greist.)

Classic Mahogany PapulesLupus Pernio Associated With Chronic Fibrotic SarcoidosisPruritic Annular Lesions

Lupus Pernio Associated With Chronic Fibrotic Sarcoidosis

A 43-year-old man admitted to the hospital had a 15-year history of arthritis. He complained of painless swelling of the proximal interphalangeal joints of the second, third, and fourth fingers of the left hand (A) and pernio-like, violaceous swellings of both large toes. He also had a localized thickening of the skin on his forehead-a sharply demarcated, painless, violaceous plaque that had gradually developed over the years (B).

X-ray films of the man’s hands and feet showed osteitis fibrosa cystica, and chest films revealed bilateral hilar lymphadenopathy. A Kveim test was positive for sarcoidosis, and skin biopsy of the forehead plaque showed noncaseating epithelioid cell granuloma intermingling with lymphocytes and Langhans giant cells. Cultures were negative for bacteria and fungi.

Lupus pernio is associated with other forms of chronic fibrotic sarcoidosis, including upper respiratory tract involvement, bone cysts, and renal sarcoidosis. Chronic sarcoid arthritis may begin early or late in the course of the disease. In rare cases, it may have an insidious onset and produce polyarticular involvement suggestive of rheumatoid arthritis.

Skin lesions of more than 2 years’ duration, as seen in this patient, seldom resolve.

(Case and photographs courtesy of Drs N. K. Akritidis, C. Tolis, and J. Goudovenos.)

Classic Mahogany PapulesLupus Pernio Associated With Chronic Fibrotic SarcoidosisPruritic Annular Lesions

Pruritic Annular Lesions

This 38-year-old man had a 4-year history of generalized annular, pruritic, copper brown lesions. An extensive workup revealed cutaneous sarcoidosis.

Papules and plaques are the most common cutaneous manifestations of sarcoidosis. In this patient, the plaques are in an annular configuration. Lesions with a strong vascular prominence are called angiolupoid.

Any patient with sarcoidosis should undergo a complete workup so that the systems involved may be identified. Although there is no cure, corticosteroids are commonly used for systemic involvement or severe cutaneous symptoms. Antimalarial agents have also been used for the treatment of recalcitrant cutaneous disease.

(Case and photograph courtesy of Dr Charles E. Crutchfield III.)