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November 17, 2008

Sweet-like Dermatosis in 2 Patients With Clinical Features of Dermatomyositis and Underlying Autoimmune Disease

Author Affiliations

Author Affiliations: Departments of Dermatology (Drs Owen, Malone, and Callen) and Pathology (Dr Malone), University of Louisville, Louisville, Kentucky.

Arch Dermatol. 2008;144(11):1486-1490. doi:10.1001/archderm.144.11.1486

Background  The neutrophilic dermatoses comprise a group of cutaneous disorders that are characterized histopathologically by infiltration of the dermis with mature neutrophils with or without vessel wall destruction. Neutrophilic dermatoses have been reported in association with a variety of autoimmune diseases, most recently as a manifestation of lupus erythematosus.

Observations  We describe 2 patients with photodistributed violaceous plaques: one with associated heliotrope rash and malar erythema, and the other with scalp involvement and Gottron-like papules. In each case, the biopsy specimen revealed changes compatible with a neutrophilic dermatosis as opposed to an interface dermatitis. The first patient also had a history of Graves disease and primary biliary cirrhosis, while second patient had Wegener granulomatosis. The 2 patients responded to therapy with oral dapsone and prednisone, respectively.

Conclusions  The atypical presentation of neutrophilic dermatosis in 2 patients with clinical features of dermatomyositis and intercurrent autoimmune-mediated illnesses may suggest an expansion in the clinical spectrum of parainflammatory neutrophilic dermatoses. The finding of a neutrophilic dermatosis in a biopsy specimen from a patient without a classic clinical presentation should invoke a thoughtful search for underlying immune complex–mediated systemic disease.