Scleromyxedema
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《新英格兰医药杂志》
A 70-year-old patient presented with a one-year history of progressive tightening of the skin. Physical examination showed generalized sclerodermoid induration and discoloration of the skin (Panel A), as well as decreased motility of the mouth and joints. Serum electrophoresis showed a monoclonal gammopathy of the IgG type, with lambda light chains. Imaging showed pleural and pericardial effusions and decreased motility of the esophagus. Examination of a deep-skin biopsy specimen showed fibrosis, proliferation of fibroblasts, and interstitial deposits of mucin in the dermis (Panel B). A diagnosis of scleromyxedema was made. The patient died three months later owing to respiratory and cardiac failure. Scleromyxedema is a primary dermal mucinosis that is almost always associated with a monoclonal gammopathy. Progression to myeloma is rare.
Stefan Schanz, M.D.
Gerhard Fierlbeck, Ph.D.
University Tübingen
D-72076 Tübingen, Germany
Stefan Schanz, M.D.
Gerhard Fierlbeck, Ph.D.
University Tübingen
D-72076 Tübingen, Germany