Clearing of epidermolysis bullosa acquisita with cyclosporine

Abstract Epidermolysis bullosa acquisita is a chronic, severe, subepidermal, blistering disease of the skin, characterized by marked resistance to topical and systemic therapy. This report concerns a well-documented case of a woman who had had epidermolysis bullosa acquisita for 6 years and had remained hospitalized continuously for 7 months in 1987. Her case ultimately was controlled with cyclosporine after the failure of a variety of therapeutic modalities in the hospital, including prednisone, methotrexate, azathioprine, phenytoin, vitamin E, gold sodium thiomalate (Myochrysine), isotretinoin, and plasmapheresis. In contrast to patients with pemphigus and pemphigoid treated with cyclosporine, our patient's autoantibodies did not disappear on therapy. Although its mechanism of action in epidermolysis bullosa acquisita is unknown, we propose that cyclosporine may be a helpful drug for patients whose disease is refractory to more traditional forms of therapy.