Pemphigus Foliaceus

  • Laura de Sena Nogueira MaeharaEmail author
  • Marcel F. Jonkman


Pemphigus foliaceus (PF) is a group of autoimmune skin blistering diseases comprising four subtypes: Cazenave, endemic, erythematosus, and herpetiformis. The common characteristic is the frequent presence of pathogenic anti-desmoglein 1 antibodies leading to bullae in the skin. Other characteristics – clinical, histological, pathogenesis, and therapy – vary and are specified in this chapter.

Pemphigus foliaceus Cazenave first described by Cazenave is the classical form of PF. The elemental dermatological lesions are erythematous plaques with pastry puff squames predominantly affecting seborrheic areas. The histopathological hallmark is acantholysis in the upper part of the epidermis (subcorneal or intraspinous). The disease is caused by autoantibodies to desmoglein 1 on the epithelial cell surface. Therapy is based on systemic corticosteroids and immunosuppressive adjuvants. Rituximab and other anti-CD20 biologics targeting B lymphocytes have changed the prognosis of the disease from chronic relapsing to long-term remissions.

Endemic pemphigus foliaceus is a variant of PF that is endemic under peasants in rural Brazil, Indian tribes and gold miners in Colombia, and under women in Tunisia. The histopathology, immunology, and therapy are similar to PF Cazenave.


Desmosome Immunoglobulin Autoimmune disease Vesiculobullous disease Pemphigus 


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Further Reading

  1. Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, Mimouni D, Borradori L, Feliciani C, Ioannides D, Joly P, Kowalewski C, Zambruno G, Zillikens D, Jonkman MF. Pemphigus. S2 guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2014;29:405–14.CrossRefPubMedGoogle Scholar

On the Web

  1. International Pemphigus & Pemphigoid Foundation
  2. JAMA Dermatology Patient Page, Pemphigus

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Laura de Sena Nogueira Maehara
    • 1
    • 2
    Email author
  • Marcel F. Jonkman
    • 2
  1. 1.Department of DermatologyPaulista School of Medicine, Federal University of São Paulo, Hospital São PauloSão PauloBrazil
  2. 2.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

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