Intravenous immunoglobulin treatment in therapy-resistant epidermolysis bullosa acquisita

J Am Acad Dermatol. 1997 Feb;36(2 Pt 2):331-5. doi: 10.1016/s0190-9622(97)80411-2.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

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

Substances

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