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Related Experiment Videos

Scleromyxedema

A M Dinneen1, C H Dicken

  • 1Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.

Journal of the American Academy of Dermatology
|July 1, 1995
PubMed
Summary
This summary is machine-generated.

Scleromyxedema, a rare fibromucinous disorder, has a poor prognosis and is difficult to treat. While alkylating agents may offer short-term benefits for scleromyxedema, long-term use presents significant toxicity.

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Area of Science:

  • Dermatology
  • Oncology
  • Internal Medicine

Background:

  • Scleromyxedema is a rare fibromucinous disorder.
  • Associated with significant morbidity and mortality.
  • Treatment challenges are common.

Purpose of the Study:

  • To investigate the natural history of scleromyxedema.
  • To evaluate the response to alkylating agent therapy.

Main Methods:

  • Clinicopathologic review of 26 scleromyxedema patients.
  • Noted extracutaneous findings.
  • Assessed response to alkylating agents.

Main Results:

  • Extracutaneous manifestations (e.g., gastrointestinal) occurred in 20/26 patients.
  • Abnormal paraproteins (most commonly IgG-lambda) found in 23/26.
  • Melphalan therapy in 17 patients resulted in 10 fatalities.

    • Conclusions:

    • Scleromyxedema carries a poor overall prognosis.
    • Therapy for scleromyxedema is challenging.
    • Alkylating agents show short-term benefit but long-term toxicity.