Sweet-like dermatosis in 2 patients with clinical features of dermatomyositis and underlying autoimmune disease.
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.


METHODS
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.
Owen
Cindy England
CE
Department of Dermatology, University of Louisville, Louisville, KY 40202, USA. ceowen01@louisville.edu
Malone
Janine C
JC
Callen
Jeffrey P
JP
D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D001707:Biopsy, Needle; D003622:Dapsone / Q000627:therapeutic use; D003882:Dermatomyositis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology; D006111:Graves Disease / Q000175:diagnosis / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D012008:Recurrence; D018570:Risk Assessment; D012720:Severity of Illness Index; D016463:Sweet Syndrome / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D016896:Treatment Outcome
D003622:Dapsone