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Endemic pemphigus foliaceus or fogo selvagem (<jats:styled-content style=\"fixed-case\">FS<\/jats:styled-content>) is one of the variants of pemphigus foliaceus pemphigus foliaceus that shares the same clinical and immunopathological features of the classic non\u2010endemic pemphigus foliaceus form, including pathogenic <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content> (mainly <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>4) autoantibodies directed against the ectodomain of desmoglein 1 (<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>sg1), that lead to acantholysis. Pathogenesis of <jats:styled-content style=\"fixed-case\">FS<\/jats:styled-content> is complex, involving genetic, environmental and immunological factors. Human leukocyte antigen (<jats:styled-content style=\"fixed-case\">HLA)\u2010DRB<\/jats:styled-content>1 alleles <jats:styled-content style=\"fixed-case\">DRB<\/jats:styled-content>1*0404, *1402, *1406 or *0102 have been previously identified as risk factors for <jats:styled-content style=\"fixed-case\">FS<\/jats:styled-content> (relative risk, &gt;14). Individuals exposed to hematophagous insects are more susceptible to develop the disease. Non\u2010pathogenic anti\u2010<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>sg1 antibodies of the <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>1 subclass, directed against the extracellular 5 domain of <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>sg1, are detected in patients in the preclinical stage of the disease, and also in healthy controls living in endemic areas. In counterpart, patients with <jats:styled-content style=\"fixed-case\">FS<\/jats:styled-content> show pathogenic anti\u2010<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>sg1 <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>g<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>4 autoantibodies that bind the pathogenic extracellular 1 and 2 domains of <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>sg1, emphasizing the intramolecular epitope\u2010spreading hypothesis. A possible explanation for the development of the autoimmune process would be antigenic mimicry, initiated by environmental stimuli in those genetically predisposed individuals. Characterization of the pathogenesis of <jats:styled-content style=\"fixed-case\">FS<\/jats:styled-content> will allow the development of specific therapeutic targets, and the elucidation of other autoimmune processes.<\/jats:p>","DOI":"10.1111\/1346-8138.12675","type":"journal-article","created":{"date-parts":[[2015,1,5]],"date-time":"2015-01-05T10:11:10Z","timestamp":1420452670000},"page":"18-26","source":"Crossref","is-referenced-by-count":77,"title":["Update on fogo selvagem, an endemic form of pemphigus foliaceus"],"prefix":"10.1111","volume":"42","author":[{"given":"Valeria","family":"Aoki","sequence":"first","affiliation":[{"name":"Department of Dermatology University of S\u00e3o Paulo Medical School S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evandro A.","family":"Rivitti","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of S\u00e3o Paulo Medical School S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis A.","family":"Diaz","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Cooperative Group on Fogo Selvagem Research","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,1,5]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.390"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(89)70079-7"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep13070394"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0733-8635(18)30140-2"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2011.03.014"},{"key":"e_1_2_7_7_1","unstructured":"VieiraJP.Novas contribui\u00e7\u00f5es ao estudo do p\u00eanfigo foli\u00e1ceo end\u00eamico (fogo selvagem) no Estado de S\u00e3o Paulo: Empresa Gr\u00e1fica da Revista dos Tribunais;1940."},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12298213"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1990.tb01446.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1590\/abd1806-4841.20142679"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12029"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1988.tb02590.x"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2009.03265.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(96)90832-4"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13088"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4632.2012.05725.x"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.2012.04468.x"},{"issue":"2","key":"e_1_2_7_18_1","first-page":"59","article-title":"P\u00eanfigo folliaceus end\u00eamico na inf\u00e2ncia: estudo evolutivo \/ Endemic pemphigus foli\u00e1ceo in children: study of the evaluation","volume":"61","author":"Castro RM","year":"1986","journal-title":"An Bras Dermatol"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.03.020"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4362.1970.tb04592.x"},{"key":"e_1_2_7_21_1","unstructured":"Aranha\u2010Campos ed.P\u00eanfigo foli\u00e1ceo (fog selvagem): aspectos cl\u00ednicos e epidemiol\u00f3gicos: Companhia Melhoramentos;1942."},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0738-081X(99)00014-0"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2006.07302.x"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(95)91330-0"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12456932"},{"issue":"2","key":"e_1_2_7_26_1","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1093\/jmedent\/35.2.120","article-title":"Comparison of black fly species (Diptera: Simuliidae) on an Amerindian reservation with a high prevalence of fogo selvagem to neighboring disease\u2010free sites in the State of Mato Grosso do Sul, Brazil. 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