Intravenous immunoglobulin treatment in therapy-resistant epidermolysis bullosa acquisita.
Epidermolysis bullosa acquisita is an uncommon autoimmune bullous disease of the skin and mucous membranes. It is chronic, disabling, and difficult to treat. We describe a case of severe epidermolysis bullosa acquisita of 7 years' duration that had been treated with azathioprine, corticosteroids, chlorambucil, plasma exchanges, cyclophosphamide, cyclosporine, and colchicine without any lasting effect. Seven cycles of treatment were administered with immunoglobulin given intravenously at a low dose, 40 mg/kg body weight daily for 5 days. The patient was free of disease for 10 months after the initiation of therapy. We suggest that low-dose regimens of immunoglobulins may be as effective in this disease as the high-dose regimens suggested in the literature, and at much lower cost.
Kofler
H
H
Department of Dermatology, University of Innsbruck, Austria.
Wambacher-Gasser
B
B
Topar
G
G
Weinlich
G
G
Schuler
G
G
Hintner
H
H
Romani
N
N
Fritsch
P
P
D000328:Adult; D003176:Complement C3 / Q000032:analysis; D004351:Drug Resistance; D016107:Epidermolysis Bullosa Acquisita / Q000473:pathology / Q000628:therapy*; D005340:Fibrinogen / Q000032:analysis; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008856:Microscopy, Fluorescence; D009055:Mouth / Q000473:pathology; D012074:Remission Induction
D003176:Complement C3; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D005340:Fibrinogen