Current concepts in the treatment of epidermolysis bullosa acquisita

Abstract

Introduction: Long-term remission in EBA patients is difficult to achieve. Patients who are resistant or develop side effects to conventional immunosuppressive therapy (CIST) have been treated with several other agents.

Areas covered: This review focuses on the clinical outcome in patients treated with a single drug or combination, and determines if long-term remission can be induced. Data on 71 patients was analyzed. There are no controlled trials. The regimens used included dapsone, colchicine, mesalazine, cyclosporine, mycophenolic acid, intravenous immunoglobulin, rituximab, daclizumab, extracorporeal photochemotherapy, and plasmapheresis. An algorithm on treating a patient has been presented.

Expert opinion: The use of CIST, especially in wide spread and recalcitrant patients, usually does not produce a prolonged clinical remission and can have hazardous side effects. Intravenous immunoglobulin, rituximab and immunoadsorption have been successfully used in some, but the benefits from their use may require additional studies. The immediate future holds much promise for the development of a specific ELISA which may facilitate the early diagnosis of EBA. As the population ages, it is likely that the number of patients will increase and newer biological agents may emerge that may have a better clinical outcome. One of the challenges is to produce “targeted therapies”.

Keywords::

• colchicine
• cyclosporine
• daclizumab
• dapsone
• epidermolysis bullosa acquisita
• extracorporeal photochemotherapy
• intravenous immunoglobulin
• mesalazine
• mycophenolic acid
• plasmapheresis
• rituximab

Acknowledgements

The authors are grateful to O Lyczmanenko, library manager, and C Vaillancourt of the Woodard Library of the New England Baptist Hospital for their assistance in obtaining the necessary literature.

Notes

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