{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T17:49:29Z","timestamp":1779904169836,"version":"3.53.1"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2018,4,14]],"date-time":"2018-04-14T00:00:00Z","timestamp":1523664000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Generalized pustular psoriasis (<jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>) is a potentially life\u2010threatening disease that can be attributed to mutations in <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content><\/jats:italic> in a subgroup of patients. In small trials, interleukin (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>)\u201017A and <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201017<jats:styled-content style=\"fixed-case\">RA<\/jats:styled-content> antagonists have been shown to be effective in patients with generalized pustular psoriasis in Japan. We identified seven patients who received the <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201017A antagonists secukinumab (six cases) or ixekizumab (one case) in two dermatological centers. All patients showed a good or excellent clinical response. Anti\u2010<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201017A therapy was well tolerated and ongoing in all patients after an average therapy duration of 12.9 months. Analysis of <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content><\/jats:italic> mutation status was performed in six patients, one patient carried a heterozygous mutation, while the other five patients did not show a mutation in <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content><\/jats:italic>. This is the first report of a successful treatment of <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> patients without <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content><\/jats:italic> mutations responding to anti\u2010<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201017A therapy.<\/jats:p>","DOI":"10.1111\/1346-8138.14318","type":"journal-article","created":{"date-parts":[[2018,4,14]],"date-time":"2018-04-14T23:02:04Z","timestamp":1523746924000},"page":"850-854","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Successful treatment with interleukin\u201017A antagonists of generalized pustular psoriasis in patients without <i>IL36RN<\/i> mutations"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5594-7192","authenticated-orcid":false,"given":"Dagmar","family":"Wilsmann\u2010Theis","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergy University of Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa Marie","family":"Schnell","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy University of Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronika","family":"Ralser\u2010Isselstein","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy University of Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Bieber","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy University of Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"Sch\u00f6n","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center G\u00f6ttingen G\u00f6ttingen Germany"},{"name":"Lower Saxony Institute of Occupational Dermatology University Medical Center G\u00f6ttingen and University of Osnabr\u00fcck G\u00f6ttingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"H\u00fcffmeier","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Friedrich\u2010Alexander\u2010Universit\u00e4t Erlangen\u2010N\u00fcrnberg Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rotraut","family":"M\u00f6ssner","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center G\u00f6ttingen G\u00f6ttingen 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