Scleromyxedema: a complete response to prednisone.

Abstract:

:Scleromyxedema is a disorder characterized by a typical rash due to the accumulation of mucin in the dermis. It is always associated with a monoclonal protein in the serum and can have a wide variety of systemic manifestations. We describe a 40-year-old woman who had scleromyxedema associated with a monoclonal G lambda protein. Severe systemic symptoms included fatigue, esophageal dysmotility, and myopathy. Symptoms resolved completely with oral prednisone therapy, and she remained in clinical remission 24 months after use of prednisone was discontinued. Scleromyxedema is commonly treated with alkylating agents, which have been associated with pronounced morbidity and mortality. We suggest that oral corticosteroid therapy may be a reasonable initial choice for treating this disease and that alkylating agents be reserved for corticosteroid-refractory disease.

journal_name

Mayo Clin Proc

journal_title

Mayo Clinic proceedings

authors

Rayson D,Lust JA,Duncan A,Su WP

doi

10.4065/74.5.481

keywords:

subject

Has Abstract

pub_date

1999-05-01 00:00:00

pages

481-4

issue

5

eissn

0025-6196

issn

1942-5546

pii

S0025-6196(11)65128-8

journal_volume

74

pub_type

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