{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,11]],"date-time":"2026-11-11T01:28:02Z","timestamp":1794360482873,"version":"4.9.0"},"reference-count":21,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,2,14]],"date-time":"2017-02-14T00:00:00Z","timestamp":1487030400000},"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":["Int J Dermatology"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Limited information exists regarding juvenile generalized pustular psoriasis (<jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>). We aim to determine the clinical profile and outcome of Malaysians with juvenile <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Review of hospital case notes on patients with juvenile <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010seven patients with juvenile <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> were identified. Female to male ratio was 1.4:1. The median age at onset of <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> was 6.5 years. Ten patients had prior psoriasis with a median pre\u2010pustular duration of 2.7 years. Onset of <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, <jats:italic>P<\/jats:italic> = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> was present in six and one patient(s), respectively. Twenty\u2010one patients had acute, five annular, and one localized variant of <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content> variants and one has <jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14 mutation. <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content>\u2010positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long\u2010term systemic therapy. Acitretin and cyclosporine were effective in aborting acute <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> was controlled with systemic agents were successfully weaned off treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Juvenile <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> is a chronic recalcitrant disease. <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>36<jats:styled-content style=\"fixed-case\">RN<\/jats:styled-content>\u2010positive patients have more severe disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijd.13489","type":"journal-article","created":{"date-parts":[[2017,2,14]],"date-time":"2017-02-14T08:42:41Z","timestamp":1487061761000},"page":"392-399","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia"],"prefix":"10.1111","volume":"56","author":[{"given":"Bi\u2010Wen","family":"Lau","sequence":"first","affiliation":[{"name":"School of Medicine and Health Sciences Monash University Melbourne Vic. Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dee\u2010Zhen","family":"Lim","sequence":"additional","affiliation":[{"name":"School of Medicine and Health Sciences Monash University Melbourne Vic. Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Capon","sequence":"additional","affiliation":[{"name":"Division of Genetics and Molecular Medicine King\u2019s College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan N.","family":"Barker","sequence":"additional","affiliation":[{"name":"Division of Genetics and Molecular Medicine King\u2019s College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siew\u2010Eng","family":"Choon","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah Johor Bahru Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,2,14]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2009.09593.x"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2009.07.029"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1525-1470.2001.018003188.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2007.02014.x"},{"key":"e_1_2_7_6_1","first-page":"144","article-title":"Generalized pustular psoriasis. 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