Cutaneous Necrotizing Sarcoidosis

Abstract

A 33‐year‐old African American woman presented with a 2 year history of skin lesions on her legs. Skin biopsy demonstrated interstitial and focally confluent reticular dermal histiocytic infiltrate consistent with granuloma annulare. She was treated with high‐potency topical steroids and returned 14 months later with diffuse papular lesions, 80 lb. weight loss, and dyspnea. Repeat skin biopsy showed similar distribution of interstitial and sheet‐like histiocytopathy, accompanied by fibrinoid necrosis, cell‐poor lymphocytic interface dermatitis and thrombogenic vasculopathy. Stains for fungi (AB – PAS and GMS) and mycobacterial species (Ziehl‐Neelsen, Fite and auramine‐rhodamine immunofluorescence) were negative. No foreign material was evident under polarized light. A diagnosis of cutaneous necrotizing sarcoidosis was made. Necrotizing sarcoidosis is a well‐recognized pulmonary entity, but is rarely reported in the skin. Diagnostic features include granulomata with regional necrosis, often with a granulomatous vasculitis, in the absence of demonstrable microbial pathogens. Patients are typically adult women who present with pulmonary symptoms, fever, weight loss and fatigue. The etiology of sarcoidosis remains obscure. One proposed mechanism is an id reaction to an as yet unidentified pathogen, with circulating microbial antigens leading to an occlusive and infarctive vasculopathy. Such a mechanism could explain the pathologic changes seen with our patient.