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Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examinations, is essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected by both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay (ELISA). Before the introduction of corticosteroids, pemphigus vulgaris was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids, with azathioprine, dapsone, methotrexate, cyclophosphamide, and gold as adjuvants or alternatives, but mycophenolate mofetil and intravenous immunoglobulins also appear promising. <\/jats:p>","DOI":"10.1177\/154411130201300504","type":"journal-article","created":{"date-parts":[[2009,1,9]],"date-time":"2009-01-09T00:40:32Z","timestamp":1231461632000},"page":"397-408","source":"Crossref","is-referenced-by-count":109,"title":["P<scp>emphigus<\/scp> V<scp>ulgaris:<\/scp> U<scp>pdate on<\/scp> E<scp>tiopathogenesis,<\/scp> O<scp>ral<\/scp> M<scp>anifestations, and<\/scp> M<scp>anagement<\/scp>"],"prefix":"10.1177","volume":"13","author":[{"given":"Crispian","family":"Scully","sequence":"first","affiliation":[{"name":"Department of Oral Medicine, Eastman Dental Institute for Oral Health Care Sciences, University College London, University of London, 256 Gray\u2019s Inn Road, London WC1X 8LD, UK;"},{"name":"Department of Oral and Maxillofacial Medicine and Pathology, Guy\u2019s Kings and Thomas\u2019 Medical and Dental Schools, Kings College, University of London, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Challacombe","sequence":"additional","affiliation":[{"name":"Department of Oral Medicine, Eastman Dental Institute for Oral Health Care Sciences, University College London, University of London, 256 Gray\u2019s Inn Road, London WC1X 8LD, UK;"},{"name":"Department of Oral and Maxillofacial Medicine and Pathology, Guy\u2019s Kings and Thomas\u2019 Medical and Dental Schools, Kings College, University of London, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2002,9,1]]},"reference":[{"key":"AHMED-AND-HOMBAL-1987","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(87)70226-6"},{"key":"AHMED-AND-MOY-1982","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(82)70111-2"},{"key":"AHMED-ETAL-1980","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-92-3-396"},{"key":"AHMED-ETAL-1990","unstructured":"Ahmed AR, Yunis EJ, Khatri K, Wagner R, Notani G, Awdeh Z, et al. 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