Autoimmune hepatitis is a subtle diagnosis that has many diverse clinical presentations. It has been reported in the literature to occur concomitantly with pyoderma gangrenosum, a neutrophilic dermatosis. Sweet’s syndrome is another neutrophilic dermatosis and has been reported to be associated with autoimmune hepatitis in only 2 previous cases: 1 idiopathic and 1 drug induced. Here we report a third case in a 24-year-old woman diagnosed with Sweet’s syndrome in association with autoimmune hepatitis, documenting a possible trend between neutrophilic dermatoses and autoimmune hepatitis. The patient presented with a history of fever and tender, erythematous plaques on her legs. Skin biopsy of a plaque confirmed histiocytoid Sweet’s syndrome. Initial laboratory investigations revealed elevated transaminases, and liver biopsy confirmed autoimmune hepatitis. This case suggests autoimmune hepatitis should be considered as an association when investigating a patient with Sweet’s syndrome.
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