Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Pemphigus Vulgaris, Esophageal

  • Joaninha Costa RosaEmail author
  • Ana Afonso
Reference work entry


Pemphigus vulgaris


Pemphigus vulgaris (PV) is a rare, chronic, life-threatening, autoimmune, blistering disease of the skin and mucosal membranes. It results from the loss of integrity of normal intercellular attachments within epithelia, producing suprabasal acantholytic blisters. The separation of the epithelial cells at their desmosomal junctions is due to the production of autoantibodies targeting epithelial adhesion molecules.

The disorder may have a complex etiology involving genetic and environmental factors. The susceptibility to pemphigus is described as being related to several human leukocyte antigen (HLA) alleles and to ethnic groups.

The disease is commonly associated with other immune disorders and with an increase in the incidence of neoplasia, but it is distinct from the variant paraneoplastic pemphigus.

In a majority of cases, pemphigus vulgarisstarts in the oral mucosa, but it may also occur in a variety of other mucosal sites. After the onset of...

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References and Further Reading

  1. Calonje, E., Brenn, T., Lazar, A., & McKee, P. H. (2012). Acantholytic disorders. In E. Calonje, T. Brenn, A. Lazar, & P. H. McKee (Eds.), McKee’s pathology of the skin (pp. 151–179). Edinburgh: Elsevier.CrossRefGoogle Scholar
  2. Faias, S., Lage, P., Sachse, F., Pinto, A., Fidalgo, P., Fonseca, I., & Nobre-Leitão, C. (2004). Pemphigus vulgaris with exclusive involvement of the esophagus: Case report and review. Gastrointestinal Endoscopy, 60(2), 312–315.CrossRefGoogle Scholar
  3. Hokama, A., Yamamoto, Y., Taira, K., Nakamura, M., Kobashigawa, C., Nakamoto, M., Hirata, T., Kinjo, N., Kinjo, F., Takahashi, K., & Fujita, J. (2010). Esophagitis dissecans superficialis and autoimmune bullous dermatosis: A review. World Journal of Gastrointestinal Endoscopy, 2(7), 252–256.CrossRefGoogle Scholar
  4. Stanley, J. R., & Amagai, M. (2006). Pemphigus, bullous impetigo and the staphylococcal scalded- skin syndrome. The New England Journal of Medicine, 355, 1800–1810.CrossRefGoogle Scholar
  5. Zhang, X., Hyjek, E., Soltani, K., Petronic-Rosic, V., & Shea, C. R. (2012). Immunohistochemistry for immunoglobulin g4 on paraffin sections for the diagnosis of pemphigus. Archives of Pathology and Laboratory Medicine, 136(11), 1402–1407.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Serviço de Anatomia PatológicaInstituto Português de Oncologia de Lisboa Francisco GentilLisbonPortugal
  2. 2.Serviço de Anatomia PatológicaHospital Cuf Descobertas e IPOLFG, E.P.E Parque das NaçõesLisbonPortugal