{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T21:52:29Z","timestamp":1790545949462,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,2,25]],"date-time":"2024-02-25T00:00:00Z","timestamp":1708819200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001003","name":"Boehringer Ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>GPP is a rare, chronic, neutrophilic skin disease, with limited real\u2010world data characterizing patients with flares and the impact of flares on disease progression and morbidity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Describe the clinical characteristics of patients with GPP, comorbidities, disease epidemiology and frequency and severity of flares, and compare patients with GPP with a matched severe psoriasis population.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this population\u2010based real\u2010world cohort study an algorithm was developed to identify patients with GPP flares. Three cohorts were identified using the Syst\u00e8me National des Donn\u00e9es de Sant\u00e9 (SNDS) database covering almost the entire French population; a prevalent cohort (2010\u20132018), an incident cohort (2012\u20132015). A severe psoriasis cohort was compared with the GPP incident cohort using propensity score matching.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The prevalent and incident cohorts comprised 4195 and 1842 patients, respectively. In both cohorts, mean age was 58\u2009years; 53% were male. Comorbidities were significantly more common in the incident cohort versus matched psoriasis cohort, respectively, including hypertension (44% vs. 26%), ischaemic heart disease (26% vs. 18%) and hyperlipidaemia (25% vs. 15%). In the incident cohort, the flare rate was 0.1 flares\/person\u2010year and 0.4 flares\/person\u2010year among the 569 out of 1842 patients hospitalized with flares. These patients had a mean (\u00b1SD) stay of 11.6\u2009\u00b1\u200910.4\u2009days; 25% were admitted to the intensive care unit. In 2017, the cumulative incidence and cumulative GPP age\u2013sex standardized prevalence were 7.1 and 45.2 per million, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with GPP had a distinct comorbidity profile compared to patients with severe psoriasis, and GPP flares were associated with long hospitalizations.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.19901","type":"journal-article","created":{"date-parts":[[2024,2,26]],"date-time":"2024-02-26T07:34:04Z","timestamp":1708932844000},"page":"1131-1139","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Generalized pustular psoriasis: A nationwide population\u2010based study using the National Health Data System in France"],"prefix":"10.1111","volume":"38","author":[{"given":"Manuelle","family":"Viguier","sequence":"first","affiliation":[{"name":"Department of Dermatology\u2010Venereology H\u00f4pital Robert Debr\u00e9 and Universit\u00e9 Reims\u2010Champagne Ardenne, IRMAIC, EA 7509  Reims France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malek","family":"Bentayeb","sequence":"additional","affiliation":[{"name":"Cerner Enviza  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Azzi","sequence":"additional","affiliation":[{"name":"Cerner Enviza  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rard","family":"de Pouvourville","sequence":"additional","affiliation":[{"name":"ESSEC Business School  Cergy\u2010Pontoise France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tristan","family":"Gloede","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim Pharmaceuticals, Inc  Ridgefield Connecticut USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9reng\u00e8re","family":"Langellier","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Massol","sequence":"additional","affiliation":[{"name":"Aixial Consulting  Boulogne Billancourt France"},{"name":"Remede Consulting Group  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Medina","sequence":"additional","affiliation":[{"name":"Cerner Enviza  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Thoma","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim International GmbH  Biberach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herv\u00e9","family":"Bachelez","sequence":"additional","affiliation":[{"name":"Service de Dermatologie, Assistance Publique\u2013H\u00f4pitaux de Paris H\u00f4pital Saint\u2010Louis, and INSERM Unit\u00e9 1163, Imagine Institute of Genetic Diseases, Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,2,25]]},"reference":[{"key":"e_1_2_11_2_1","first-page":"131","article-title":"Pustular psoriasis: pathophysiology and current treatment perspectives","volume":"6","author":"Benjegerdes KE","year":"2016","journal-title":"Psoriasis (Auckl)"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.14386"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-3388"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1968.tb11947.x"},{"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":"10.1111\/ijd.12070"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16084"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.14523"},{"issue":"6","key":"e_1_2_11_10_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_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.12863"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.20966"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2021.4640"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555766871"},{"issue":"2","key":"e_1_2_11_15_1","first-page":"173","article-title":"Clinical analysis of 82 cases of generalized pustular psoriasis","volume":"42","author":"Wu X","year":"2017","journal-title":"Zhong Nan Da Xue Xue Bao Yi Xue Ban"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1991.01680080075005"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/pds.4233"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.16566"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2020.2977"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-8587(19)30191-3"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.respe.2017.05.004"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.therap.2018.09.072"},{"key":"e_1_2_11_23_1","unstructured":"Caisse nationale de l'Assurance Maladie des travailleurs salari\u00e9s. Am\u00e9liorer la qualit\u00e9 du syst\u00e8me de sant\u00e9 et ma\u00eetriser les d\u00e9penses: propositions de l'Assurance Maladie pour 2016.2015. Available from:https:\/\/www.vie\u2010publique.fr\/sites\/default\/files\/rapport\/pdf\/275504.pdf?msclkid=d9dd9debd14e11ec8b17787315195e19. Accessed 27 July 2022."},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1177\/24755303211021779"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1159\/000529274"},{"key":"e_1_2_11_26_1","unstructured":"eurostat. Database.2022. Available from:https:\/\/ec.europa.eu\/eurostat\/data\/database. Accessed 27 July 2022."},{"key":"e_1_2_11_27_1","unstructured":"Institut National de la Statistique et des \u00c9tudes Economiques. Les r\u00e9sultats des recensements de la population.2022. Available from:https:\/\/www.insee.fr\/fr\/information\/2008354. Accessed 27 July 2022."},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18720"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.09.049"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13555-021-00661-2"},{"key":"e_1_2_11_31_1","unstructured":"Fran\u00e7aisR.Les d\u00e9penses de sant\u00e9en 2018. R\u00e9sultats des comptes de la sant\u00e9 \u00e9dition 2019.2019. Available from:https:\/\/dreessolidarites\u2010santegouvfr\/sites\/default\/files\/2020\u201007\/cns2019.pdf. Accessed 27 July 2022."},{"key":"e_1_2_11_32_1","unstructured":"European Medicines Agency.Development of medicines for rare diseases. Available from:https:\/\/www.ema.europa.eu\/en\/news\/development\u2010medicines\u2010rare\u2010diseases#:~:text=A%20disease%20is%20considered%20rare means%20one%20in%2017%20people. Accessed 27 July 2022."},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/cpt.329"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2021.06.008"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2111563"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(23)01378-8"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.1093\/bjd\/ljad083"}],"container-title":["Journal of the European Academy of Dermatology and Venereology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jdv.19901","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,25]],"date-time":"2024-05-25T21:21:51Z","timestamp":1716672111000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jdv.19901"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2,25]]},"references-count":36,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,6]]}},"alternative-id":["10.1111\/jdv.19901"],"URL":"https:\/\/doi.org\/10.1111\/jdv.19901","archive":["Portico"],"relation":{},"ISSN":["0926-9959","1468-3083"],"issn-type":[{"value":"0926-9959","type":"print"},{"value":"1468-3083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2,25]]},"assertion":[{"value":"2023-05-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-02-25","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}