Pathology of the Gastrointestinal Tract

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

Pemphigoid, Bullous, Esophageal

  • Ana AfonsoEmail author
  • Joaninha Costa Rosa
Reference work entry


Anti-epiligrin cicatricial pemphigoid; Anti-laminin 5 cicatricial pemphigoid; Bullous pemphigoid; Cicatricial pemphigoid; Mucous membrane pemphigoid


Esophageal pemphigoid is an uncommon disorder belonging to a group of autoimmune blistering diseases that can affect the mucosa, hence the designation of mucous membrane pemphigoid (MMP). It was formerly named cicatricial pemphigoid because, in the majority of the patients, the lesions have a tendency to heel with a scar. Bullous pemphigoid (BP) is nowadays the preferred term for the bullous disease limited to the skin, where it may initially start as an urticarial eruption that progressively evolves to large and tense bullae. BP rarely affects mucosal membranes. Skin involvement in MMP is seen in 20–25% of patients (Sallout et al. 2000); in 10% of the cases, skin lesions are the first sign of the disease, but they are usually less severe than in BP. MMP has the singular quality of healing with scar formation, and this...

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References and Further Reading

  1. Bernard, P., & Borradori, L. (2012). Pemphigoid group. In J. L. Bolognia, J. L. Jorizzo, & J. V. Schaffer (Eds.), Dermatology (pp. 475–490). Philadelphia: Elsevier/Saunders.Google Scholar
  2. Bruch-Gerharz, D., Hertl, M., & Ruzicka, T. (2007). Mucous membrane pemphigoid: Clinical aspects, immunopathological features and therapy. European Journal of Dermatology, 17, 191–200.PubMedGoogle Scholar
  3. Calonje, E., Brenn, T., Lazar, A., & McKee, P. H. (2012). Inherited and autoimmune subepidermal blistering diseases. In E. Calonje, T. Brenn, A. Lazar, & P. H. McKee (Eds.), MacKee’s pathology of the skin (pp. 133–137). Edinburgh: Elsevier Limited.Google Scholar
  4. Chan, L. S., Ahmed, R. A., Anhalt, G. J., Bernauer, W., Cooper, K. D., Elder, M. J., Fine, J. D., Foster, S., Ghohestani, R., Hashimoto, T., Hoang-Shuan, T., Kirtschig, G., Korman, N. J., Lightman, S., Lozada-Nur, F., Marinkovich, P., Mondino, B. J., Prost-Squarcioni, C., Rogers, R. S., Setterfield, J. F., West, D. P., Wojnarowska, F., Woodley, D. T., Yancey, K. B., Zillikens, D., & Zone, J. J. (2002). The first international consensus on mucous membrane pemphigoid. Definition, diagnostic criteria, pathogenic factors, medical treatment and prognostic indicators. Archives of Dermatology, 138, 370–379.PubMedGoogle Scholar
  5. Sallout, H., Anhalt, G., & Al-Kawas, F. (2000). Mucous membrane pemphigoid presenting with isolated esophageal involvement: A case report. Gastrointestinal Endoscopy, 52, 429–433.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

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