{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T14:24:42Z","timestamp":1749133482182},"reference-count":27,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,4,2]],"date-time":"2015-04-02T00:00:00Z","timestamp":1427932800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2015,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pemphigus is an autoimmune blistering disease caused by immunoglobulin (Ig)G autoantibodies against desmogleins (Dsg). In mucosal\u2010dominant pemphigus vulgaris (<jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>), anti\u2010Dsg3 antibodies play a critical role in acantholysis. We followed two mucosal\u2010dominant <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> cases who suffered from refractory oral mucosal erosions. In these cases, anti\u2010Dsg3 serum antibodies were not detected by indirect immunofluorescence and enzyme\u2010linked immunosorbent assay (<jats:styled-content style=\"fixed-case\">ELISA<\/jats:styled-content>). However, direct immunofluorescence showed the intercellular IgG deposition in the epidermis and histopathological findings revealed suprabasal acantholysis. In order to analyze the pathomechanisms in these cases, we first examined the Dsg3 expression patterns in lesional sites and compared them with those of typical mucosal\u2010dominant <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> cases. In typical <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> cases, the alteration of Dsg3 distribution was observed in lesional sites by immunostaining. The aggregation of Dsg3, which is the characteristic change in <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> mucosal lesions, was observed as the initial change prior to acantholysis. In our cases, a clustering of Dsg3 was observed at mucosal lesions, and the expression levels of Dsg3 in acantholytic lesions were decreased, as observed in typical mucosal\u2010dominant <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> cases. Although anti\u2010Dsg3 serum antibodies could not be detected by routine tests, anti\u2010Dsg3 serum antibodies were detected by Dsg3 <jats:styled-content style=\"fixed-case\">ELISA<\/jats:styled-content> using 10\u2010times more concentrated sera (highly sensitive <jats:styled-content style=\"fixed-case\">ELISA<\/jats:styled-content>). Moreover, purified and concentrated <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> IgG showed high pathogenicity when examined by dissociation assay. In conclusion, the detection of morphological changes in Dsg3 distribution and highly sensitive <jats:styled-content style=\"fixed-case\">ELISA<\/jats:styled-content> method could be useful for the early diagnosis of <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> recurrence.<\/jats:p>","DOI":"10.1111\/1346-8138.12872","type":"journal-article","created":{"date-parts":[[2015,4,2]],"date-time":"2015-04-02T22:15:06Z","timestamp":1428012906000},"page":"572-579","source":"Crossref","is-referenced-by-count":5,"title":["Clues to diagnosis for unusual mucosal pemphigus demonstrating undetectable anti\u2010desmoglein 3 serum antibodies by routine tests"],"prefix":"10.1111","volume":"42","author":[{"given":"Koji","family":"Kamiya","sequence":"first","affiliation":[{"name":"Department of Dermatology Hamamatsu University School of Medicine Hamamatsu Japan"},{"name":"Department of Dermatology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yumi","family":"Aoyama","sequence":"additional","affiliation":[{"name":"Department of Dermatology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mari","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Department of Dermatology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aya","family":"Ukida","sequence":"additional","affiliation":[{"name":"Department of Dermatology Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuko","family":"Mizuno\u2010Ikeda","sequence":"additional","affiliation":[{"name":"Department of Dermatology Okayama University 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