{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:49:51Z","timestamp":1787014191726,"version":"build-2736575974"},"reference-count":26,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,3,3]],"date-time":"2023-03-03T00:00:00Z","timestamp":1677801600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2023,8]]},"abstract":"Abstract<\/jats:title>\n \n Generalized Pustular psoriasis (GPP), a rare and potentially life\u2010threatening auto\u2010inflammatory disease, is associated with\n IL36RN<\/jats:italic>\n mutations. Here, we analyse the prevalence of\n IL36RN<\/jats:italic>\n mutations in our multi\u2010ethnic GPP cohort and assess differences in the clinical profile of patients with (\n IL36RN<\/jats:italic>\n \u2010positive) and without (\n IL36RN\u2010<\/jats:italic>\n negative) mutations.\n IL36RN<\/jats:italic>\n mutations were present in 17.7% of 137 GPP patients (29.7% of Chinese cases, 17.3% of Malay cases, but 0% of Indian patients). 92% of these individuals carried the c.115\u2009+\u20096\u00a0T\u2009>\u2009C mutation. Male: female ratio was 1:2.3. Females predominate in both groups with no significant difference between\n IL36RN\u2010<\/jats:italic>\n positive and\n IL36RN<\/jats:italic>\n \u2010negative individuals. The overall mean age (\u00b1SD) at disease onset for GPP was 37.6\u2009\u00b1\u200917.2\u2009years, but disease onset was significantly earlier in\n IL36RN<\/jats:italic>\n \u2010positive vs\n IL36RN<\/jats:italic>\n \u2010negative cases (mean age:30.6\u2009\u00b1\u200918.92 vs. 39.2\u2009\u00b1\u200916.49\u2009years,\n p<\/jats:italic>\n \u00a0=\u00a00.027).\n IL36RN\u2010<\/jats:italic>\n positive patients were less likely to have associated plaque psoriasis (52.4% vs. 83.5%,\n p<\/jats:italic>\n \u2010value\u00a0=\u00a00.002). There was no difference in the common clinical and laboratory manifestations or triggers of GPP between\n IL36RN\u2010<\/jats:italic>\n positive and \u2010negative patients, except for geographic tongue which was significantly more common in\n IL36RN\u2010<\/jats:italic>\n positive patients (41.7% vs. 11.9%,\n p<\/jats:italic>\n \u2010value\u00a0=\u00a00.002). Annual flare rate was significantly higher in\n IL36RN\u2010<\/jats:italic>\n positive compared to\n IL36RN<\/jats:italic>\n \u2010negative (mean\u2009\u00b1\u2009SD of 1.92\u2009\u00b1\u20091.32 vs. 1.46\u2009\u00b1\u20090.90,\n p<\/jats:italic>\n \u00a0=\u00a00.041) cases. However, no significant difference in the rate of hospitalization and length of hospital stay was observed between the two groups. These observations demonstrate that\n IL36RN<\/jats:italic>\n disease alleles occur with varying frequencies among Asian populations and are associated with a severe, early\u2010onset clinical phenotype.\n <\/jats:p>","DOI":"10.1111\/exd.14776","type":"journal-article","created":{"date-parts":[[2023,2,26]],"date-time":"2023-02-26T22:19:25Z","timestamp":1677449965000},"page":"1263-1271","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Clinical profile of patients with acute generalized pustular psoriasis with and without\n IL36RN<\/scp>\n mutations in multi\u2010ethnic Johor Bahru, Malaysia"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7796-5746","authenticated-orcid":false,"given":"Siew Eng","family":"Choon","sequence":"first","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah Johor Bahru Johor Bahru Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences Monash University Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter Seah Keng","family":"Tok","sequence":"additional","affiliation":[{"name":"Institute for Clinical Research, National Institutes of Health, Ministry of Health Malaysia Putrajaya Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kit Wan","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah Johor Bahru Johor Bahru Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yee Ting","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah Johor Bahru Johor Bahru Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nalini M.","family":"Nanu","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah Johor Bahru Johor Bahru Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan N.","family":"Barker","sequence":"additional","affiliation":[{"name":"St John's Institute of Dermatology at Guys and St Thomas's Hospitals and Kings College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2432-5793","authenticated-orcid":false,"given":"Francesca","family":"Capon","sequence":"additional","affiliation":[{"name":"Department of Medical and Molecular Genetics King's College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,3,3]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2019.1648209"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-021-00654-z"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.14386"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.12070"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2019.1708193"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2021.06.008"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22169048"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.07.022"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2014.09.043"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.3892\/ijmm.2021.4951"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2018.06.038"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.230"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.214"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2012.490"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.267"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.25259\/IJDVL_1054_2021"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-015-1611-x"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.13034"},{"key":"e_1_2_11_21_1","first-page":"669","article-title":"Generalized pustular psoriasis (Zumbusch): a French epidemiological survey","volume":"16","author":"Augey F","year":"2006","journal-title":"Eur J Dermatol"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555766871"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdin.2021.12.001"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.20966"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000023"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2111563"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-016-1750-y"}],"container-title":["Experimental 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