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.
Maize
John C
JC
Department of Dermatology, University of Texas, Southwestern Medical School, Dallas, 75390, USA.
Cohen
Jack B
JB
D000328:Adult; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004820:Epidermolysis Bullosa / Q000150:complications / Q000188:drug therapy*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D012008:Recurrence; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D016572:Cyclosporine