How is scleromyxedema treated

How is scleromyxedema treated

What therapy options exist for scleromyxedema?

Historically, therapies used in multiple myeloma (melphalan, cyclophosphamide) have been described with variable success in scleromyxedema, but also come with the risk of significant side effects. Additionally, the cutaneous disease can progress even with eradication of the monoclonal paraprotein, as the skin course does not necessarily track with the gammopathy. More recent case series have described successful use of intravenous immunoglobulin (IVIG). Given its favorable side-effect profile in comparison with chemotherapy regimens, IVIG should be considered for initial therapy. Successful treatment often requires maintenance IVIG, as relapses off therapy have been described. Corticosteroid and thalidomide use have also been described in case reports with some success.

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