INTRAVENOUS IMMUNOGLOBULIN TREATMENT IN THERAPY-RESISTANT EPIDERMOLYSIS-BULLOSA ACQUISITA

Epidermolysis bullosa acquisita is an uncommon autoimmune bullous dise ase of the skin and mucous membranes. It is chronic, disabling, and di fficult to treat. We describe a case of severe epidermolysis bullosa a cquisita of 7 years' duration that had been treated with azathioprine, corticosteroids, chlorambucil, plasma exchanges, cyclophosphamide, cy closporine, and colchicine without any lasting effect. Seven cycles of treatment were administered with immunoglobulin given intravenously a t a low dose, 40 mg/kg body weight daily for 5 days. The patient was f ree of disease for 10 months after the initiation of therapy. We sugge st that low-dose regimens of immunoglobulins may be as effective in th is disease as the high-dose regimens suggested in the literature, and at much lower cost.