Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema.

Scleromyxedema or generalized lichen myxedematosus is a rare depositional disorder. Diagnostic criteria encompass a generalized papular and sclerodermoid eruption, monoclonal gammopathy (paraproteinemia), most often with G-lambda type immunoglobulin, a characteristic microscopic triad (mucin deposition, fibroblast proliferation, fibrosis), and absence of thyroid disease. Many internal manifestations of scleromyxedema have been described to date, leading to high mortality and morbidity. Because the disease is rare, the etiology is not fully understood and there is a lack of well-designed studies, so no optimal treatment exists so far. This paper reports the follow-up on a patient in 5.5-year remission after successful intravenous immunoglobulin therapy 10.5 years since initial diagnosis.