Mucous membrane pemphigoid
- Mucous membrane pemphigoid
DiseaseDisorder infobox
Name = Pemphigoid
ICD10 = L12
ICD9 = ICD9|694
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.
Presentation
In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin, specifically at the level of the basement membrane, which connects the lower skin layer (dermis) to the upper skin layer (epidermis) and keeps it attached to the body.
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 it 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.
Diagnosis
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.
ee also
* Bullous pemphigoid
* Gestational pemphigoid
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