Disease Information for Pemphigoid, cicatricial

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Clinical Manifestations
Signs & Symptoms
Baldness/Alopecia
Blisters
Bulla/bullous rash
Cicatrical alopecia
Hair loss
Associated Diseases & Rule outs
Rule Outs
Behcet's syndrome
Pemphigoid, bullous
Pemphigus
Associated Disease & Complications
Scarring alopecia
Disease Mechanism & Classification
Class
CLASS/Dermatologic/Subcutaneous (category)
Process
PROCESS/Reference organ/system (category)
Definition

A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring; It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement; ---------------------------------------.Mucous Membrane Pemphigoid; CP; Cicatricial Pemphigoid; MMP; Mucous Membrane Pemphigoid ; Subdivisions: Brunsting-Perry Syndrome; Localized Cicatricial Pemphigoid; Vegetating Cicatricial Pemphigoid; Mucous membrane pemphigoid (MMP) is a rare group of chronic autoimmune disorders characterized by blistering lesions that primarily affect the various mucous membranes of the body; The mucous membranes of the mouth and eyes are most often affected; The mucous membranes of the nose, throat, genitalia, and anus may also be affected; The symptoms of MMP vary among affected individuals depending upon the specific site(s) involved and the progression of the disease; Blistering lesions eventually heal, sometimes with scarring; Progressive scarring may potentially lead to serious complications affecting the eyes and throat; In some cases, blistering lesions also form on the skin, especially in the head and neck area; The exact cause of MMP is unknown; Mucous membrane pemphigoid has been known by many different names within the medical literature including benign mucous membrane pemphigoid, cicatricial (scarring) pemphigoid, and ocular cicatricial pemphigoid; In March of 2002, a consensus group of researchers determined that mucous membrane pemphigoid was the best designation for this group of disorders; The term "benign" mucous membrane pemphigoid was deemed inappropriate because of the potential for serious complications in some cases; The term "cicatricial" pemphigoid excluded affected individuals who do not develop scarring; Site-specific terms such as "ocular" cicatricial pemphigoid excluded individuals with multiple site involvement;[NORD]

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NEJM (The New England Journal of Medicine)
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