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Genetic and Rare Diseases Information Center (GARD)

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Cicatricial pemphigoid


Other Names for this Disease

  • Benign mucosal pemphigoid
  • Benign mucous membrance pemphigoid
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Overview

Cicatricial pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. The condition usually begins in late adulthood (e.g. 50's or 60's), affects more women than men, and has a variable prognosis.[1] Scarring of the affected  mucosa of the eye may lead to blindness and tends to be the most feared complication.[2] A combination of environmental and genetic factors appear to play a role in the susceptibility of developing cicatricial pemphigoid.[1] Although the specific causes of this condition have not been identified, it is considered an autoimmune disease that is characterized by the production of autoantibodies against basement membrane zone antigens such as BP180, BP230, and laminin 5.[2][3]  Treatment is dependent on the person's specific symptoms.
Last updated: 11/14/2014

References

  1. Bruch-Gerharz D, Hertl M, Ruzicka T. Mucous membrane pemphigoid: clinical aspects, immunopathological features and therapy. Eur J Dermatol. 2007 May-Jun; 17(3):191-200. http://www.ncbi.nlm.nih.gov/pubmed/?term=Bruch-Gerharz+D%2C+Hertl+M%2C+Ruzicka+T+2007. Accessed 11/14/2014.
  2. Pemphigoid. McPherson & Pincus: Henry's Clinical Diagnosis and Management by Laboratory Methods, 21st ed. Pennsylvania: W.B. Saunders Company; 2006;
  3. Torchia D, Caproni M, Volpi W, Fabbri P. Naturally occurring regulatory T cells in mucous membrane pemphigoid lesions. Acta Dermatoven APA. 2009 Mar; 18(1):3-6. http://s3-eu-west-1.amazonaws.com/thejournalhub/10.15570/archive/acta-apa-09-1/1.pdf. Accessed 11/14/2014.
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Other Names for this Disease
  • Benign mucosal pemphigoid
  • Benign mucous membrance pemphigoid
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.