[Scleromyxedema: immunosuppressive therapy with cyclophosphamide].

A 59-year-old male with scleromyxedema showed lichenoid papules over the large joints and buttocks; excessive skin folds, and generalized sclerosis of the skin similar to scleroderma; myopathy and arthritis; cardiovascular and cerebrovascular disease; and an abnormal monoclonal IgG-type protein in the serum. The progression of the disease, lack of established therapeutic modalities and the pronounced systemic involvement in this condition suggested a therapeutic trial employing monthly high-dose i.v. cyclophosphamide pulse therapy, together with low-dose prednisone on alternate days. The results were excellent and both subjective and objective symptoms improved dramatically. Controls performed at regular time intervals during a follow-up period of 12 months have failed to reveal any recurrence of the disease.