Intractable epidermolysis bullosa acquisita: efficacy of cyclosporin A.
We report the case of a 66-year-old man who presented with a spontaneous blistering involving both skin and mucous membranes, diagnosed as an inflammatory form of epidermolysis bullosa acquisita. During a 5-year course, the condition failed to respond to various therapies. Finally, only ciclosporin, together with a low dose regimen of prednisone, achieved a dramatic improvement with a total clearing within 6 months, persisting 1 1/2 years after discontinuation. Such results prompt us to propose such an association as a primary therapeutic scheme in an otherwise barely tractable disease.
Department of Dermatology, Hôpital St. Jacques, Besançon, France.
C
C
Merle
D
D
Blanc
M
M
Zultak
H
H
van Landuyt
C
C
Drobacheff
R
R
Laurent
D000368:Aged; D003524:Cyclosporins / Q000008:administration & dosage / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D004820:Epidermolysis Bullosa / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D012867:Skin / Q000648:ultrastructure
D003524:Cyclosporins; D011241:Prednisone