PEMPHIGUS FOLIACEUS TREATED SUCCESSFULLY WITH PLASMA-EXCHANGE

A 64-year-old man had suffered from pemphigus foliaceus since 1979. The clinical diagnosis of pemphigus foliaceus was confirmed by histologic and immunologic examinations of a skin biopsy. Over the years he had several relapses despite treatment with steroids, erythromycin, gold, azathioprine, and cyclosporine. In 1989, the skin lesions became more severe even though prednisone and cyclophosphamide was increased (Fig. 1), and the patient developed severe side effects such as pneumonia, hypertension, heart failure, and noninsulin dependent diabetes mellitus. Serum titer of intercellular antibodies (ICAb) was 1:10,000 when plasma exchange was initiated. Four to 5 L were exchanged with albumin daily for 3 days. The skin symptoms disappeared, and the prednisone and the antihypertensive could be withdrawn. Six months later the patient had a full-blown relapse. Serum titer of ICAb was 1:2,500. Again treatment with high-dose cyclophosphamide and prednisone was given, but without success; the patient developed severe side effects. Plasma exchanges were resumed and continued for 2.5 years with an interval of 6 weeks. The skin lesions rapidly improved. The serum level of ICAb decreased to 1:600. Prednisone was lowered to 7.5 mg/day and cyclophosphamide withdrawn. At present the patient is in remission and continues with plasma exchanges every second month.