Scleromyxedema with dermato-neuro syndrome

Scleromyxedema is a rare connective tissue disease of unknown cause charact erized by a generalized papular eruption, dermal fibroblast proliferation, and monoclonal paraproteinemia. A paroxysmal triad consisting of high fever , seizures, and coma with a flu-like prodrome can rarely occur in patients with scleromyxedema and is termed "dermato-neuro syndrome." We describe a 4 1-year-old patient with scleromyxedema in whom the dermato-neuro syndrome d eveloped.