We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor VIII deficiency.
- Authors
Maize, Jr, John C.; Cohen, Jack B.
- Abstract
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. Herein, we report a challenging case of EBA and acquired factor VIII deficiency in the same patient. 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. 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.
- Subjects
EPIDERMOLYSIS bullosa; CYCLOSPORINE; NONSTEROIDAL anti-inflammatory agents; IMMUNOSUPPRESSIVE agents; BLISTERS; BLOOD coagulation factor VIII antibodies
- Publication
International Journal of Dermatology, 2005, Vol 44, Issue 8, p692
- ISSN
0011-9059
- Publication type
Other
- DOI
10.1111/j.1365-4632.2004.02245.x