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Pemphigus vulgaris : ウィキペディア英語版 | Pemphigus vulgaris Pemphigus vulgaris is a chronic blistering skin disease with skin lesions that are rarely pruritic, but which are often painful.〔Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.〕 It is classified as a type II hypersensitivity reaction, with the formation of anti-desmosome antibodies. ==Pathophysiology== It is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes resulting in the loss of desmosomes and therefore the loss of cohesion between keratinocytes in the epidermis, classified as a type II hypersensitivity reaction (in which antibodies bind to antigens on the body's own tissues). It is characterized by extensive flaccid blisters and mucocutaneous erosions. The severity of the disease, as well as the mucosal lesions, is believed to be directly proportional to the levels of desmoglein 3. Milder forms of pemphigus (like foliacious and erythematoses) are more desmoglein 1 heavy. It arises most often in middle-aged or older people, usually starting with a blister that ruptures easily. It can also start with blisters in the mouth. The lesions can become quite extensive. The pathogenesis of the disease involves autoantibodies against desmosome proteins, separating keratinocytes from the basal layer of the epidermis. On histology, the basal keratinocytes are usually still attached to the basement membrane leading to the appearance and thus the term, "tombstoning".
Transudative fluid accumulates in between the keratinocytes and basement membrane (suprabasal split), forming a blister and resulting in what is known as a positive Nikolsky's sign. This is a contrasting feature from bullous pemphigoid, which is thought to be due to anti-hemidesmosome antibodies, and where the detachment occurs between the epidermis and dermis (subepidermal bullae).
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