Scleromyxedema and the dermato‐neuro syndrome: case report and review of the literature

Scleromyxedema is a generalized form of lichen myxedematosus which is characterized by a papular and sclerodermoid skin eruption resulting from dermal fibroblast proliferation and mucin deposition. The majority of patients with scleromyxedema have a monoclonal gammopathy, and other systemic manifestations are common. Herein we describe a case of the ‘dermato‐neuro syndrome’, a rare and sometimes fatal neurologic manifestation of scleromyxedema which consists of fever, convulsions and coma, often preceded by a flu‐like prodrome. In addition, we provide a comprehensive summary of previously published cases of the dermato‐neuro syndrome and discuss the current etiopathogenic theories and treatment options for this rare disease. Fleming KE, Virmani D, Sutton E, Langley R, Corbin J, Pasternak S, Walsh NM. Scleromyxedema and the dermato‐neuro syndrome: case report and review of the literature.