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|MedlinePlus = 000882 |eMedicineSubj = derm |eMedicineTopic = 317 |eMedicine_mult = ~ foliaceus Drug-induced ~ ~ herpetiformis ~ IgA ~ Vulgaris ~Paraneoplastic Benign ~ (Hailey-Hailey Disease) |MeshID = D010392 }} Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.〔 〕 In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.〔(International Pemphigus & Pemphigoid Foundation: What is Pemphigus? )〕 Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separates to form the blisters. In 1971, an article investigating the autoimmune nature of this disease was published. ==Types== There are several types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, Intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus. *Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack ''Desmoglein 3''. Sores often originate in the mouth, making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management. 〔Abreu-Velez AM, Calle-Isaza J, Howard MS. Autoimmune epidermal blistering diseases. Our Dermatol Online. 2013;4(Suppl.3):631-646〕 * Pemphigus foliaceus (PF) is the least severe of the three varieties. ''Desmoglein 1'', the protein that is destroyed by the autoantibody, is found in only the top dry layer of the skin. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema 〔Abreu Velez AM, Calle-Isaza J, Howard MS. Autoimmune epidermal blistering diseases. Our Dermatol Online. 2013;4(Suppl.3):631-646. DOI: 10.7241/ourd.20134.158〕 Endemic pemphigus foliaceus, including the Fogo Selvagem, the new variant of endemic pemphigus folaiceus in El Bagre, Colombia, South America, and the Tunisian endemic pemphigus in North Africa.〔1. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Pemphigus」の詳細全文を読む スポンサード リンク
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