Treatment of epidermolysis bullosa acquisita with cyclosporine and prednisone

Abstract

A case of epidermolysis bullosa acquisita in a 58-year-old woman is described. The diagnosis was made on the basis of the clinical picture as well as the histopathological, immunopathological and ultrastructural findings. The patient was treated for over 4 years with corticosteroids, azathioprine, etretinate and dapsone; these drugs produced only partial remissions of short duration. Furthermore, the patient developed many severe side-effects induced by corticosteroids. For these reasons, treatment was started with cyclosporine (5 mg/kg per day) and prednisone (5 mg/day), resulting in complete remission of cutaneous lesions within 3 months. The dosage was subsequently reduced by 0.5 mg/kg per day every 6 months. After 3 years and 6 months the patient was still under treatment with cyclosporine (3 mg/kg per day) and prednisone (5 mg/day). No relevant side-effects induced by cyclosporine had been seen. This case suggests that cyclosporine, combined with prednisone, can be efficacious in the treatment of epidermolysis bullosa acquisita even at low dosages.