Cicatricial Pemphigoid

Common Name(s)

Cicatricial Pemphigoid

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. 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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Cicatricial Pemphigoid" for support, advocacy or research.

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International Pemphigus and Pemphigoid Foundation

The IPPF provides direct access to innovative and effective support that promotes the very best health care, improves quality of life, stimulates community resources, advocates for favorable government policies, accelerates the pace of scientific discovery and is the world’s best source of information on pemphigus and pemphigoid. We are compassionate in understanding, tireless in service, and relentless in advocating. One person at a time, we make a difference, building a community of care and hope.

Last Updated: 8 Mar 2013

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Cicatricial Pemphigoid" for support, advocacy or research.

Logo
International Pemphigus and Pemphigoid Foundation

The IPPF provides direct access to innovative and effective support that promotes the very best health care, improves quality of life, stimulates community resources, advocates for favorable government policies, accelerates the pace of scientific discovery and is the world’s best source of information on pemphigus and pemphigoid. We are compassionate in understanding, tireless in service, and relentless in advocating. One person at a time, we make a difference, building a community of care and hope.

http://www.pemphigus.org/

Last Updated: 8 Mar 2013

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Cicatricial Pemphigoid" returned 61 free, full-text research articles on human participants. First 3 results:

[A particular type of cicatricial Pemphigoid with unique IgA deposit].
 

Author(s): Amina Aounallah, Mariem Jrad, Mehdi Ksiaa, Sana Mokni, Wafa Saidi, Lobna Boussofara, Badreddine Sriha, Mohamed Denguezli, Najet Ghariani, Colandane Belajouza, Rafia Nouira

Journal:

 

Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic ...

Last Updated: 31 Dec 1969

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NGF Modulates trkANGFR/p75NTR in αSMA-Expressing Conjunctival Fibroblasts from Human Ocular Cicatricial Pemphigoid (OCP).
 

Author(s): Alessandra Micera, Barbara Stampachiacchiere, Antonio Di Zazzo, Roberto Sgrulletta, Magdalena Cortes, Eduardo Maria Normando, Alessandro Lambiase, Stefano Bonini

Journal:

 

In a previous study, we reported the upregulation of Nerve Growth Factor (NGF) and trkANGFR expression in Ocular Cicatricial Pemphigoid (OCP), an inflammatory and remodeling eye disease. Herein, we hypothesize a potential NGF-driven mechanism on fibroblasts (FBs) during OCP remodeling ...

Last Updated: 31 Dec 1969

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Neutrophil collagenase, gelatinase, and myeloperoxidase in tears of patients with stevens-johnson syndrome and ocular cicatricial pemphigoid.
 

Author(s): Samer N Arafat, Ana M Suelves, Sandra Spurr-Michaud, James Chodosh, C Stephen Foster, Claes H Dohlman, Ilene K Gipson

Journal: Ophthalmology. 2014 Jan;121(1):79-87.

 

To investigate the levels of matrix metalloproteinases (MMPs), myeloperoxidase (MPO), and tissue inhibitor of metalloproteinase-1 (TIMP-1) in tears of patients with Stevens-Johnson syndrome (SJS) and ocular cicatricial pemphigoid (OCP).

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Cicatricial Pemphigoid" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Last Updated: 22 Sep 2017

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Last Updated: 12 Jun 2017

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Clinical Trial on the Effect of Autologous Oral Mucosal Epithelial Sheet Transplantation
 

Status: Available

Condition Summary: Limbal Stem Cell Deficiency; Stevens-johnson Syndrome; Ocular Cicatricial Pemphigoid; Chemical Burn

 

Last Updated: 18 Apr 2016

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