Scleromyxedema revisited.
Scleromyxedema is a rare disease characterized by papular mucinous deposits, dermal fibroblast proliferation, and a monoclonal paraproteinemia. Its chronic course tends to be complicated by gastrointestinal, muscular, pulmonary, and neurologic disorders. We review the literature to identify the common systemic manifestations and suggest a clinical approach to this disorder. This should include: identification of the characteristic lesions, a skin biopsy, and serum protein electrophoresis. A thyroid evaluation may help differentiate this condition from others. An early neurologic, gastrointestinal, and cardiovascular evaluation should be considered.
Pomann
John J
JJ
Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA.
Rudner
Earl J
EJ
D000368:Aged; D001707:Biopsy, Needle; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009230:Myxedema / Q000453:epidemiology* / Q000473:pathology* / Q000503:physiopathology; D011379:Prognosis; D035583:Rare Diseases; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000453:epidemiology* / Q000473:pathology* / Q000503:physiopathology; D012720:Severity of Illness Index; D014481:United States / Q000453:epidemiology