Mucous membrane pemphigoid
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| Pemphigoid Classification and external resources | |
| ICD-10 | L12 |
|---|---|
| ICD-9 | 694 |
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Mucous membrane pemphigoid, also known as cicatricial pemphigoid (which means scarring pemphigoid) is a skin-blistering autoimmune disease. This is a rare condition with an incidence of approximately one in a million of the population.
In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin specifically at the level of the basement membrane, the layer that holds the skin to the underlying tissue. When the condition is active, the basement membrane is dissolved by the antibodies produced, and areas of skin lift away at the base, causing hard blisters which scar if they burst. In other words, this is a desquamating/blistering disease in which the epithelium "unzips" from the underlying connective tissue, allowing fluid to gather that subsequently manifest as bullae, or blisters.
The autoimmune reaction most commonly affects the mouth, causing lesions in the gingiva or gums, but can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals and anus. When the cornea of the eye is affected, repeated scarring may result in blindness.
Diagnostic techniques: antibodies (IgG) precipitates complement (C3) in the lamina lucida of the basement membrane. Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2. Positive Nikolsky sign. IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
