TY - JOUR
T1 - Fatal scleromyxedema
T2 - Report of a case and review of the literature
AU - Godby, A.
AU - Bergstresser, P. R.
AU - Chaker, B.
AU - Pandya, A. G.
N1 - Funding Information:
This article is made possible through an educational grant from Ortho Dermatological.
PY - 1998
Y1 - 1998
N2 - 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.
AB - 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.
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U2 - 10.1016/s0190-9622(98)70567-5
DO - 10.1016/s0190-9622(98)70567-5
M3 - Article
C2 - 9486700
AN - SCOPUS:0031895305
SN - 0190-9622
VL - 38
SP - 289
EP - 294
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 2 II
ER -