Fatal scleromyxedema: Report of a case and review of the literature - 09/09/11
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. (J Am Acad Dermatol 1998;38:289-94.)
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This article is made possible through an educational grant from Ortho Dermatological. |
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0190-9622/98/$5.00 + 0 16/4/84879 |
Vol 38 - N° 2S
P. 289-294 - février 1998 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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