Scleromyxoedema--successful treatment with plasma exchange and immunosuppression

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 4 l 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.