title: Cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor VIII deficiency.
abstract: 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.
authors.affiliation: Department of Dermatology, University of Texas, Southwestern Medical School, Dallas, 75390, USA.
authors.forename: John C
authors.initials: JC
authors.lastname: Maize
authors.forename: Jack B
authors.initials: JB
authors.lastname: Cohen
mesh_terms: 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
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine