{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T00:01:48Z","timestamp":1789430508777,"version":"build-2803163510"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2023,5,10]],"date-time":"2023-05-10T00:00:00Z","timestamp":1683676800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Global Psoriasis Atlas"},{"DOI":"10.13039\/501100012331","name":"LEO Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012331","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006483","name":"AbbVie","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006483","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100022606","name":"Almirall","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100022606","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002429","name":"Amgen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002429","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004312","name":"Eli Lilly and Company","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004312","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005565","name":"Janssen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005565","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Novartis Pharma AG and UCB"},{"name":"A.K.W., D.M.A. and C.E.M.G"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>There is limited understanding of the epidemiology of generalized pustular psoriasis (GPP) internationally, with no population-based estimates of GPP in South\u00a0East Asia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To determine the incidence and prevalence of GPP in the Malaysian population and characterize its flares and trigger factors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a population-based cohort study using the Teleprimary Care database between January 2010 and December 2020. We identified 230 dermatologist-confirmed GPP cases using International Classification of Diseases, 10th revision, diagnostic codes. Annual prevalence and incidence rates were stratified by age, sex and ethnicity. We compared data regarding flares and trigger factors for patients with GPP who had associated psoriasis vulgaris (PV) with those who did not have associated PV.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The prevalence of GPP was 198 per million (267 women, 127 men) and incidence was 27.2 per million person-years [95% confidence interval (CI) 22.8\u201331.6]; 35.3 (28.4\u201342.2) per million person-years for women and 18.3 (13.1\u201323.5) per million person-years for men. Rates were higher in Chinese individuals [prevalence 271 per million; incidence 41.6 per million person-years (28.9\u201354.3)] than in the Malay population [prevalence 186; incidence 24.6 (19.4\u201329.7)] or the Indian ethnic group [prevalence 179; incidence 25.0 (13.8\u201336.3)]. Annual prevalence was consistently higher in women than in men and highest among the Chinese population, followed by the Indian and Malay populations. Overall, 67% of patients with GPP had associated PV. The prevalence and incidence of GPP without PV were lower than GPP with PV at 66 vs. 132 per million and 19.3 (95% CI 15.6\u201323.0) vs. 8.0 (95% CI 5.6\u201310.3) per million person-years, respectively. The mean age at GPP onset was 42.7\u2005years (SD 18.4). A bimodal trend in the age of GPP onset was observed, with first and second peaks at age 20\u201329\u2005years and age 50\u201359\u2005years, respectively. Disease onset was significantly earlier in patients with GPP without PV than in those with PV [mean age 37.5\u2005years (SD 20.7) vs. 44.9\u2005years (SD 17.0), P = 0.026]. Flares occurred more frequently in patients without PV than in those with PV [mean number of flares per patient per year was 1.35 (SD 0.77) vs. 1.25 (SD 0.58), P = 0.039]. Common triggers of flares in patients with GPP who did not have PV were infections, pregnancy, menstruation and stress, whereas withdrawal of therapy, particularly systemic corticosteroids, was a more frequent trigger in patients with GPP who also had PV.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our findings contribute to the global mapping of GPP, which will help inform the management of this rare condition.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/bjd\/ljad158","type":"journal-article","created":{"date-parts":[[2023,5,11]],"date-time":"2023-05-11T00:37:25Z","timestamp":1683765445000},"page":"410-418","source":"Crossref","is-referenced-by-count":18,"title":["Incidence and prevalence of generalized pustular psoriasis in multiethnic Johor Bahru, Malaysia: a population-based cohort study using routinely captured electronic health records in the Teleprimary Care (TPC\u00ae) clinical information system from 2010 to 2020"],"prefix":"10.1093","volume":"189","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 , Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University , Selangor , Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison K","family":"Wright","sequence":"additional","affiliation":[{"name":"Centre for Pharmacoepidemiology and Drug Safety, Division of Pharmacy and Optometry, School of Health Sciences, Faculty of Biology, Medicine and Health"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher E M","family":"Griffiths","sequence":"additional","affiliation":[{"name":"Centre for Dermatology Research, NIHR Manchester Biomedical Research Centre, University of Manchester , Manchester , UK"},{"name":"Department of Dermatology, King\u2019s College Hospital, King\u2019s College London, London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kit Wan","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Hospital Sultanah Aminah , Johor Bahru , Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University , Selangor , Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kwee\u00a0Eng\u00a0","family":"Tey","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Hospital Sultanah Aminah , Johor Bahru , Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University , Selangor , Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yee Ting","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Hospital Sultanah Aminah , Johor Bahru , Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University , Selangor , Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keow Yin","family":"Chua","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Hospital Sultanah Aminah , Johor Bahru , Malaysia"},{"name":"Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University , Selangor , Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darren M","family":"Ashcroft","sequence":"additional","affiliation":[{"name":"Centre for Pharmacoepidemiology and Drug Safety, Division of Pharmacy and Optometry, School of Health Sciences, Faculty of Biology, Medicine and Health"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,5,10]]},"reference":[{"key":"2023122117352827800_ljad158-B1","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1080\/1744666X.2019.1648209","article-title":"An update on generalized pustular psoriasis","volume":"15","author":"Gooderham","year":"2019","journal-title":"Expert Rev Clin Immunol"},{"key":"2023122117352827800_ljad158-B2","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1111\/ijd.12070","article-title":"Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia","volume":"53","author":"Choon","year":"2014","journal-title":"Int J Dermatol"},{"key":"2023122117352827800_ljad158-B3","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s40257-021-00654-z","article-title":"Clinical course and characteristics of generalized pustular psoriasis","volume":"23","author":"Choon","year":"2022","journal-title":"Am J Clin Dermatol"},{"key":"2023122117352827800_ljad158-B4","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1080\/1744666X.2019.1708193","article-title":"The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review","volume":"16","author":"Kharawala","year":"2020","journal-title":"Expert Rev Clin Immunol"},{"key":"2023122117352827800_ljad158-B5","doi-asserted-by":"crossref","first-page":"1266","DOI":"10.1016\/j.jaad.2021.06.008","article-title":"Treatments and outcomes of generalized pustular psoriasis: a cohort of 1516 patients in a nationwide inpatient database in Japan","volume":"86","author":"Miyachi","year":"2022","journal-title":"J Am Acad Dermatol"},{"key":"2023122117352827800_ljad158-B6","first-page":"669","article-title":"Generalized pustular psoriasis (Zumbusch): a French epidemiological survey","volume":"16","author":"Augey","year":"2006","journal-title":"Eur J Dermatol"},{"key":"2023122117352827800_ljad158-B7","doi-asserted-by":"crossref","first-page":"68","DOI":"10.2340\/00015555766871","article-title":"Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background","volume":"76","author":"Ohkawara","year":"1996","journal-title":"Acta Derm Venereol"},{"key":"2023122117352827800_ljad158-B8","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.jdin.2021.12.001","article-title":"Generalized pustular psoriasis in Brazil: A public claims database study","volume":"6","author":"Duarte","year":"2022","journal-title":"JAAD Int"},{"key":"2023122117352827800_ljad158-B9","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1111\/bjd.20966","article-title":"Prevalence and incidence of generalized pustular psoriasis in Sweden: a population-based register study","volume":"186","author":"L\u00f6fvendahl","year":"2022","journal-title":"Br J Dermatol"},{"key":"2023122117352827800_ljad158-B10","doi-asserted-by":"crossref","first-page":"761","DOI":"10.5021\/ad.2017.29.6.761","article-title":"Prevalence of psoriasis in Korea: a population-based epidemiological study using the Korean national health insurance database","volume":"29","author":"Lee","year":"2017","journal-title":"Ann Dermatol"},{"key":"2023122117352827800_ljad158-B11","author":"Feldman"},{"key":"2023122117352827800_ljad158-B12","author":"Department of Statistics Malaysia"},{"key":"2023122117352827800_ljad158-B13","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1111\/bjd.21768","article-title":"Incidence and prevalence of psoriasis in multiethnic Johor Bahru, Malaysia: a population-based cohort study using electronic health data routinely captured in the Teleprimary Care (TPC\u00ae) clinical information system from 2010 to 2020","volume":"187","author":"Choon","year":"2022","journal-title":"Br J Dermatol"},{"key":"2023122117352827800_ljad158-B14","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1111\/jdv.14386","article-title":"European consensus statement on phenotypes of pustular psoriasis","volume":"31","author":"Navarini","year":"2017","journal-title":"J Eur Acad Dermatology Venereol"},{"key":"2023122117352827800_ljad158-B15","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1111\/jdv.18720","article-title":"Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review","volume":"37","author":"Prinz","year":"2023","journal-title":"J Eur Acad Dermatology Venereol"},{"key":"2023122117352827800_ljad158-B16","author":"Department of Statistics Malaysia"},{"key":"2023122117352827800_ljad158-B17","doi-asserted-by":"crossref","DOI":"10.3390\/ijerph20054577","article-title":"Impact of COVID-19 pandemic on healthcare utilization among patients with type 2 diabetes mellitus: a systematic review","volume":"20","author":"Amsah","year":"2023","journal-title":"Int J Environ Res Public Health"},{"key":"2023122117352827800_ljad158-B18","author":"Hing"},{"key":"2023122117352827800_ljad158-B19","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1016\/j.jaci.2018.06.038","article-title":"Clinical and genetic differences between pustular psoriasis subtypes","volume":"143","author":"Twelves","year":"2019","journal-title":"J Allergy Clin Immunol"},{"key":"2023122117352827800_ljad158-B20","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/s00403-015-1611-x","article-title":"Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients","volume":"308","author":"Wang","year":"2016","journal-title":"Arch Dermatol Res"},{"key":"2023122117352827800_ljad158-B21","first-page":"302","article-title":"A novel mutation in IL36RN underpins childhood pustular dermatosis","volume-title":"J Eur Acad Dermatol Venereol","author":"Ellingford","year":"2016"},{"key":"2023122117352827800_ljad158-B22","doi-asserted-by":"crossref","first-page":"271","DOI":"10.2165\/11586900-000000000-00000","article-title":"Clinical and laboratory features in acute generalized pustular psoriasis","volume":"12","author":"Borges-Costa","year":"2011","journal-title":"Am J Clin Dermatol"},{"key":"2023122117352827800_ljad158-B23","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1111\/ijd.13489","article-title":"Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia","volume":"56","author":"Lau","year":"2017","journal-title":"Int J Dermatol"},{"issue":"Suppl.","key":"2023122117352827800_ljad158-B24","doi-asserted-by":"crossref","first-page":"AB20","DOI":"10.1016\/j.jaad.2021.06.107","article-title":"26591 Characterization of flares in patients with generalized pustular psoriasis \u2013 a population-based study from the French National Health Data System database (SNDS)","volume":"85","author":"Bachelez","year":"2021","journal-title":"J Am Acad Dermatol"},{"key":"2023122117352827800_ljad158-B25","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1001\/jamadermatol.2022.3142","article-title":"Understanding flares in patients with generalized pustular psoriasis documented in US electronic health records","volume":"158","author":"Zema","year":"2022","journal-title":"JAMA Dermatology"},{"key":"2023122117352827800_ljad158-B26","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1007\/s13555-021-00493-0","article-title":"Unmet medical needs in the treatment and management of generalized pustular psoriasis flares: evidence from a survey of Corrona registry dermatologists","volume":"11","author":"Strober","year":"2021","journal-title":"Dermatol Ther (Heidelb)"},{"key":"2023122117352827800_ljad158-B27","first-page":"37","article-title":"Diagnosis and screening of patients with 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