Fatal scleromyxedema: Report of a case and review of the literature

A. Godby, P. R. Bergstresser, B. Chaker, A. G. Pandya

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Abstract

Scleromyxedema is a rare fibromucinous connective tissue that can be associated with systemic changes, such as myopathy, neurologic defects, esophageal dysmotility, paraproteinemia, and restrictive lung disease. We describe a fatal case of scleromyxedema in which neurologic, cardiac, gastrointestinal, and muscle changes were present. At autopsy, mucin was found in the papillary dermis of skin and in coronary and pulmonary vessels, but was absent from the brain, kidneys, heart, gastrointestinal tract, esophagus, liver, thyroid, lymph nodes, bone marrow, and pancreas. Because the pathogenesis of scleromyxedema may not always be attributable to mucin deposition, the role of circulating factors in the development of systemic manifestations warrants further investigation.

Original languageEnglish (US)
Pages (from-to)289-294
Number of pages6
JournalJournal of the American Academy of Dermatology
Volume38
Issue number2 II
StatePublished - Mar 25 1998

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ASJC Scopus subject areas

  • Dermatology

Cite this

Godby, A., Bergstresser, P. R., Chaker, B., & Pandya, A. G. (1998). Fatal scleromyxedema: Report of a case and review of the literature. Journal of the American Academy of Dermatology, 38(2 II), 289-294.