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Cicatricial pemphigoid : ウィキペディア英語版
Cicatricial pemphigoid

Cicatricial pemphigoid (also known as "Mucous Membrane Pemphigoid", "MMP", "Benign mucosal pemphigoid," "Benign mucous membrane pemphigoid,"〔 "Ocular pemphigus,"〔 and "Scarring pemphigoid") is a rare chronic autoimmune subepithelial blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement.〔Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.〕
Cicatricial pemphigoid has been referred to by a variety of designations based largely on its site of involvements, with examples of such terminology including "desquamative gingivitis," "ocular pemphigus," and "benign mucous membrane pemphigoid." However, currently "...such designations are thought to be confusing or somewhat misleadings (e.g., pemphigus in this context is a misnomer , and this disorder is hardly benign given the extent of morbidity it can cause)." 〔
==Signs and symptoms==
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.
''Localized cicatricial pemphigoid'' (also known as "Brunsting–Perry cicatricial pemphigoid"〔) refers to a localised variant of cutaneous cicatricial pemphigoid involving the head and the neck without mucosal involvement.〔
Nikolsky's sign (gentle lateral pressure) on unaffected mucosa or skin raises a bulla. If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In (Pemphigus) the epithelium tends to disintegrate rather than form a bulla.
Nikolsky's sign is present in case of Pemphigus and Cicatricial Pemphigoid but not in the case of Bullous Pemphigoid.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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