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Therapeutic Approach

Scleredema is a rare connective tissue disease of unknown etiology, which clinically presents with diffuse symmetric woody skin induration and nonpitting edema. The course of postinfectious scleredema does not seem to be affected by antibiotics. Treating the associated diseases by improving glucose control or treatment of paraproteinemia may lead to improvement of scleredema. Treatment may not be necessary unless the disease is symptomatic or causing functional issues. Our preferred agent is phototherapy.1-3 Physical therapy may be of benefit individuals with functional issues. Monoclonal gammopathy-associated scleredema often responds to intravenous immunoglobulin and/or extracorporeal photophoresis. There has been benefit shown with local radiotherapy or electron-beam irradiation. Other immunosuppressive such as methotrexate are of limited value. See Table 53-1.

Table 53-1Scleredema Treatment Table

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions ...

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