Scleromyxedema with dermato-neuro syndrome.
Scleromyxedema is a rare connective tissue disease of unknown cause characterized 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 41-year-old patient with scleromyxedema in whom the dermato-neuro syndrome developed.
Gonzalez
J
J
Section of Photopheresis and Department of Internal Medicine, Morristown Memorial Hospital, Morristown, NJ 07962-1956, USA.
Palangio
M
M
Schwartz
J
J
Klainer
A S
AS
Bisaccia
E
E
D000328:Adult; D003128:Coma / Q000209:etiology*; D005334:Fever / Q000209:etiology*; D006801:Humans; D008297:Male; D009230:Myxedema / Q000150:complications* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012640:Seizures / Q000209:etiology*; D013577:Syndrome