Mucous membrane pemphigoid 2037592 219187085 2008-06-13T23:57:16Z Invertzoo 4863204 add "the" and "to" {{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. 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. 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. ==See also== * [[Bullous pemphigoid]] * [[Gestational pemphigoid]] {{med-sign-stub}} [[Category:Oral pathology]] [[Category:Dermatology]] [[Category:Autoimmune_diseases]] [[Category:Skin_diseases]]