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: H Kofler, B Wambacher-Gasser, G Topar, G Weinlich, G Schuler, H Hintner, N Romani, P Fritsch
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 36 Issue 2 Pt 2 Pg. 331-5 (Feb 1997) ISSN: 0190-9622 [Print] United States
• PMID: 9039213 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Complement C3
• Immunoglobulin G
• Immunoglobulins, Intravenous
• Fibrinogen

Topics

• Adult
• Complement C3 (analysis)
• Drug Resistance
• Epidermolysis Bullosa Acquisita (pathology, therapy)
• Fibrinogen (analysis)
• Humans
• Immunoglobulin G (analysis)
• Immunoglobulins, Intravenous (therapeutic use)
• Male
• Microscopy, Fluorescence
• Mouth (pathology)
• Remission Induction