title: Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature.
abstract: 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.
authors.affiliation: Division of Anatomical Pathology, Department of Pathology, Capital District Health Authority and Dalhousie University, Halifax, NS, Canada.
authors.forename: Kirsten E
authors.initials: KE
authors.lastname: Fleming
authors.forename: Divya
authors.initials: D
authors.lastname: Virmani
authors.forename: Evelyn
authors.initials: E
authors.lastname: Sutton
authors.forename: Richard
authors.initials: R
authors.lastname: Langley
authors.forename: Jessica
authors.initials: J
authors.lastname: Corbin
authors.forename: Sylvia
authors.initials: S
authors.lastname: Pasternak
authors.forename: Noreen M
authors.initials: NM
authors.lastname: Walsh
mesh_terms: D049109:Cell Proliferation; D003128:Coma / Q000378:metabolism / Q000473:pathology; D020405:Dermis / Q000378:metabolism / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D009077:Mucins / Q000378:metabolism; D053718:Scleromyxedema / Q000378:metabolism / Q000473:pathology; D012640:Seizures / Q000378:metabolism / Q000473:pathology; D013577:Syndrome
chemical_list: D009077:Mucins