• J. Cutan. Pathol. · May 2012

    Review Case Reports

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

    • Kirsten E Fleming, Divya Virmani, Evelyn Sutton, Richard Langley, Jessica Corbin, Sylvia Pasternak, and Noreen M Walsh.
    • Division of Anatomical Pathology, Department of Pathology, Capital District Health Authority and Dalhousie University, Halifax, NS, Canada.
    • J. Cutan. Pathol. 2012 May 1; 39 (5): 508-17.

    AbstractScleromyxedema 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.Copyright © 2012 John Wiley & Sons A/S.

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