Scleromyxoedema—successful treatment with plasma exchange and immunosuppression

Abstract

A 34-year-old woman with scleromyxoedema failed to respond to initial treatment with cyclophosphamide or psoralen-UVA photochemotherapy (PUVA). She developed neurological symptoms which resolved after five 41 plasma exchanges performed on successive days, together with pulse methylprednisolone therapy. Subsequent treatment was with oral prednisolone and cyclophosphamide. She had had no further episodes of neurological dysfunction 8 months after plasma exchange, and the appearance of the skin had improved almost to normal. The complications and difficulty in management of scleromyxoedema are discussed.