Other Names for this Disease
- Benign mucosal pemphigoid
- Benign mucous membrance pemphigoid
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. Scarring of the affected mucosa of the eye may lead to blindness and tends to be the most feared complication. A combination of environmental and genetic factors appear to play a role in the susceptibility of developing cicatricial pemphigoid. 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. Treatment is dependent on the person's specific symptoms.Cicatricial pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular
Last updated: 11/14/2014
- 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.
- Pemphigoid. McPherson & Pincus: Henry's Clinical Diagnosis and Management by Laboratory Methods, 21st ed. Pennsylvania: W.B. Saunders Company; 2006;
- 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.
Your Questions Answeredby the Genetic and Rare Diseases Information Center
Please contact us with your questions about Cicatricial pemphigoid. We will answer your question and update these pages with new resources and 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.
- The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
- 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.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- 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.