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Pemphigus Vulgaris: An Evidence-Based Treatment Update

  • Esther Q. Wang
  • Dedee F. MurrellEmail author
Living reference work entry

Abstract

Pemphigus vulgaris is a rare autoimmune blistering disease which is associated with high mortality and morbidity. The nature of this disease necessitates long-term aggressive treatment, for which systemic corticosteroid is the gold standard. However, the serious complications associated with systemic corticosteroids have led to the development of various adjuvant therapies including azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, cyclosporine, dapsone, gold compounds, plasmapheresis, tetracyclines, and topical therapies. The evidence to support these adjuvant therapies is extremely limited and comprises of small RCTs, case series, and case studies. Newer and emerging treatments including antitumor necrosis factor agents, intravenous immunoglobulin, and rituximab appear to be promising interventions. More research should be conducted to evaluate the efficacy of these treatments.

Keywords

Pemphigus Treatment Corticosteroid Azathioprine Mycophenolate mofetil Cyclophosphamide Dapsone Rituximab Intravenous immunoglobulin RCT 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.University of New South WalesSydneyAustralia
  2. 2.Department of Dermatology at St George HospitalUniversity of New South WalesKogarah, SydneyAustralia

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