Pemphigus Herpetiformis

  • Phillip LawsEmail author
  • Neil H. Shear


Pemphigus herpetiformis is a rare variant of pemphigus first described almost 40 years ago. Clinical features overlapping with dermatitis herpetiformis but histopathologically and immunologically consistent with pemphigus are typical. Diagnosis is often delayed due to the unusual presentation and absence of blisters. Initial reports implicated antibodies to desmoglein-1 (Dsg1) as key in pathogenesis. It is increasingly clear this is not universally the case and may include Dsg-3 and/or desmocollin antibodies. Although treatment is based on small case series reports suggest a combination approach of corticosteroid and dapsone may be most appropriate with other options including azathioprine, methotrexate or mycophenolate mofetil. The response to rituximab remains to be established. This chapter provides an overview of pemphigus herpetiformis, summarises treatment options and provides a proposed strategy for management.


Pemphigus Herpetiformis Immunobullous disease Autoantibody Desmoglein 



Funding Sources


Conflict of Interest

P.M.L. None declared

N.H.S. None declared


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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyChapel Allerton Hospital, University of LeedsLeedsUK
  2. 2.Division of Dermatology, Department of Medicine (Clinical Pharmacology)Sunnybrook Health Sciences Centre/University of TorontoTorontoCanada

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