Cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor VIII deficiency.

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.