title: Intravenous immunoglobulin treatment in therapy-resistant epidermolysis bullosa acquisita.
abstract: 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.
authors.affiliation: Department of Dermatology, University of Innsbruck, Austria.
authors.forename: H
authors.initials: H
authors.lastname: Kofler
authors.forename: B
authors.initials: B
authors.lastname: Wambacher-Gasser
authors.forename: G
authors.initials: G
authors.lastname: Topar
authors.forename: G
authors.initials: G
authors.lastname: Weinlich
authors.forename: G
authors.initials: G
authors.lastname: Schuler
authors.forename: H
authors.initials: H
authors.lastname: Hintner
authors.forename: N
authors.initials: N
authors.lastname: Romani
authors.forename: P
authors.initials: P
authors.lastname: Fritsch
mesh_terms: 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
chemical_list: D003176:Complement C3; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D005340:Fibrinogen