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Background: Epidermolysis bullosa acquisita (EBA) is a rare antibody-mediated autoimmune blistering disease of adults. Likewise, acquired factor VIII deficiency is a rare antibody-mediated disease of adulthood. Both diseases can be exceedingly difficult to treat and refractory to immunomodulatory therapies., Methods: Herein, we report a challenging case of EBA and acquired factor VIII deficiency in the same patient., Results: Cyclosporine 4 mg/kg/day rapidly controlled both disease processes after the patient failed to respond to prednisone, colchicine, and pulse cyclophosphamide. The EBA relapsed when the cyclosporine was decreased to 2 mg/kg/day and the patient wore snuggly fitting new shoes, but it cleared quickly when the dose was increased. Cyclosporine has since been decreased gradually to 1.5 mg/kg/day without relapse of either condition or detectable side-effects., Conclusions: Cyclosporine should be considered when EBA and/or acquired factor VIII deficiency fail to respond to conservative therapy. Both conditions usually respond rapidly to cyclosporine 4-6 mg/kg/day.

Nineteen patients with a variety of types of epidermolysis bullosa were initially assessed in an open study of a treatment regime for the genetic type of epidermolysis bullosa. Nine of the ten patients admitted to the hospital for treatment showed definite objective improvement with decreased number of blisters and increased rate of healing of blisters. The mean percentage decrease in blister numbers from the time of admission until discharge from hospital was 76%. Sixteen patients were followed as outpatients for 5-11 months. Two patients continued to show objective improvement, while eight reported more rapid healing of their blisters. Six patients failed to improve. This treatment has a beneficial effect in inpatients with epidermolysis bullosa. The improvement may be due to the intense topical treatment rather than to a specific item of the therapy. In the long term, this treatment has little effect on the formation of new blisters, but an accelerated healing of blisters is reported by 50% of the patients.