{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T08:59:01Z","timestamp":1790499541047,"version":"4.1.0"},"reference-count":9,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2015,4,24]],"date-time":"2015-04-24T00:00:00Z","timestamp":1429833600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"the Ministry of Health, Labor and Welfare of Japan","award":["H23-028"],"award-info":[{"award-number":["H23-028"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The clinical phenotypes of pemphigus can be explained by the desmoglein (Dsg) compensation theory. However, some atypical cases such as cutaneous pemphigus vulgaris (<jats:styled-content style=\"fixed-case\">cPV<\/jats:styled-content>), in which patients have anti\u2010Dsg3 antibodies without oral erosions, do not conform to this theory. To explain the discrepancy between clinical phenotypes and anti\u2010Dsg antibody profiles, the pathogenic strength of immunoglobulin (Ig)G autoantibodies against Dsg3 must be taken into consideration. We analyzed the epitopes and blister\u2010inducing pathogenic strength of the sera from three patients having IgG against Dsg3 without oral erosions with domain\u2010swapped recombinant proteins and dissociation assay using cultured normal human epidermal keratinocytes. The results showed that all sera contained IgG directed against the amino terminal EC1 domain of Dsg3, as is found in most PV sera. However, dissociation assays revealed that the pathogenic strength of the anti\u2010Dsg3 antibodies in all three cases was extremely lower than that of typical PV cases with mucosal involvement. In conclusion, when anti\u2010Dsg3 IgG antibodies are not sufficient to inhibit the expression of Dsg3 in the oral mucosa, but can inhibit the expression in the skin, skin blisters can result. Therefore, the pathogenicity of anti\u2010Dsg3 antibodies should be regarded as a key factor contributing to the clinical phenotype in pemphigus patients with conflicting antibody profiles.<\/jats:p>","DOI":"10.1111\/1346-8138.12888","type":"journal-article","created":{"date-parts":[[2015,4,24]],"date-time":"2015-04-24T11:44:38Z","timestamp":1429875878000},"page":"685-689","source":"Crossref","is-referenced-by-count":14,"title":["Low pathogenicity of anti\u2010desmoglein 3 immunoglobulin G autoantibodies contributes to the atypical clinical phenotypes in pemphigus"],"prefix":"10.1111","volume":"42","author":[{"given":"Marwah A.","family":"Saleh","sequence":"first","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"},{"name":"Department of Dermatology Cairo University School of Medicine Cairo Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rena","family":"Hashimoto","sequence":"additional","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Kase","sequence":"additional","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"},{"name":"Central Research Laboratory Japan Blood Products Organization Kobe Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masayuki","family":"Amagai","sequence":"additional","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Yamagami","sequence":"additional","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,4,24]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra061111"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI5252"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000088513"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.2008.02871.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2005.01.106"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.448"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0022-202X.2005.23714.x"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.167.9.5439"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1523-1747.1998.00085.x"}],"container-title":["The Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2F1346-8138.12888","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1346-8138.12888","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T19:46:05Z","timestamp":1695498365000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1346-8138.12888"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,4,24]]},"references-count":9,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2015,7]]}},"alternative-id":["10.1111\/1346-8138.12888"],"URL":"https:\/\/doi.org\/10.1111\/1346-8138.12888","archive":["Portico"],"relation":{},"ISSN":["0385-2407","1346-8138"],"issn-type":[{"value":"0385-2407","type":"print"},{"value":"1346-8138","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,4,24]]}}}