TY - JOUR
T1 - Cyclosporine controls epidermolysis bullosa acquisita co-occuring with acquired factor VIII deficiency
AU - Maize, John C.
AU - Cohen, Jack B.
PY - 2005/8
Y1 - 2005/8
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=23844521057&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=23844521057&partnerID=8YFLogxK
U2 - 10.1111/j.1365-4632.2004.02245.x
DO - 10.1111/j.1365-4632.2004.02245.x
M3 - Article
C2 - 16101876
AN - SCOPUS:23844521057
SN - 0011-9059
VL - 44
SP - 692
EP - 694
JO - International Journal of Dermatology
JF - International Journal of Dermatology
IS - 8
ER -