Scleromyxoedema: Treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin

A. Kulczycki, M. Nelson, A. Eisen, M. Heffernan

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42 Scopus citations

Abstract

Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-λ paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.

Original languageEnglish
Pages (from-to)1276-1281
Number of pages6
JournalBritish Journal of Dermatology
Volume149
Issue number6
DOIs
StatePublished - Dec 1 2003

Keywords

  • Case series
  • Intravenous immunoglobulin
  • Paraprotein
  • Scleromyxoedema

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