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.