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Cicatricial pemphigoid

Other Names for this Disease
  • Benign mucosal pemphigoid
  • Benign mucous membrance pemphigoid
More Names
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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 benign mucosal pemphigoid.[1] Although the specific causes of benign mucosal pemphigoid have not been identified, the condition 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.

References

  1. Bruch-Gerharz D, Hertl M, Ruzicka T. Eur J Dermatol. 2007. http://www.john-libbey-eurotext.fr/e-docs/00/04/2F/29/vers_alt/VersionPDF.pdf. Accessed August 4, 2009.
  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. Acta Dermatoven APA. 2009. http://ibmi.mf.uni-lj.si/acta-apa/acta-apa-09-1/1.pdf. Accessed August 4, 2009.
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General Information

  • The American Academy of Oral & Maxillofacial Pathology has an information page on this topic. Click on the link above to view this information page. 
  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. Click on the link to view the information.
  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manuals Online Medical Library provides information on this condition. Click on the link to view the information.
  • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) offers printed materials on this topic. We recommend calling toll-free at 877-226-4267 to request a copy of this information.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read information on this topic.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Cicatricial pemphigoid. Click on the link to view a sample search on this topic.