{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T00:02:58Z","timestamp":1776729778752,"version":"3.51.2"},"reference-count":32,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,1,31]],"date-time":"2024-01-31T00:00:00Z","timestamp":1706659200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/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":["The Journal of Dermatology"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Generalized pustular psoriasis (GPP), a rare form of psoriasis, is characterized by neutrophil\u2010rich, sterile pustules. In Japan, GPP has intractable and rare disease designation, which allows patients to access support from national and local governments for medical expenses. Previously, similar numbers of patients in Tokyo and Hokkaido have been shown to have GPP designation, despite different population sizes. Here, we determine whether there are regional differences in the proportion of patients receiving GPP designation status in Japan and aim to identify causal factors. In this descriptive, retrospective cohort study, publicly available data were collected on the number of patients with intractable and rare disease designation for GPP in each prefecture and age classification (April 2018\u2013March 2021). Three other designated intractable and rare disease cohorts were included: pemphigus, rare skin diseases, and all diseases. The primary outcome was the standardized morbidity ratio (SMR) of patients at prefecture level (observed numbers divided by expected). Regional differences were compared with the statistical expectation for the total population and age distribution of each prefecture. Regional differences were observed in all cohorts. Overall, 1910 patients had GPP as a designated intractable and rare disease in 2020. Regional differences in SMRs for GPP were observed with high SMRs (\u22651.5) in Hokkaido, Tottori, Kagawa, and Miyazaki, and low SMRs (&lt;0.6) in Gunma and Kanagawa. Regional differences in SMRs for GPP did not correlate with the number of medical doctors or dermatologists or internal migration. The number of medical doctors or dermatologists correlated with SMRs in the rare skin diseases and total cohorts. Regional differences in Japan exist in the number of patients with GPP who have an intractable and rare disease designation. Managing rare diseases is an important public health issue, and further research is required to elucidate the factors contributing to these differences.<\/jats:p>","DOI":"10.1111\/1346-8138.17089","type":"journal-article","created":{"date-parts":[[2024,1,31]],"date-time":"2024-01-31T08:56:39Z","timestamp":1706691399000},"page":"380-390","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Regional differences in the prevalence of generalized pustular psoriasis in Japan"],"prefix":"10.1111","volume":"51","author":[{"given":"Hideki","family":"Fujita","sequence":"first","affiliation":[{"name":"Department of Dermatology Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoko","family":"Iwasaki","sequence":"additional","affiliation":[{"name":"Nippon Boehringer Ingelheim Co., Ltd  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Tsuboi","sequence":"additional","affiliation":[{"name":"Nippon Boehringer Ingelheim Co., Ltd  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoko","family":"Murashiuma","sequence":"additional","affiliation":[{"name":"Nippon Boehringer Ingelheim Co., Ltd  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5863-9315","authenticated-orcid":false,"given":"Masashi","family":"Akiyama","sequence":"additional","affiliation":[{"name":"Department of Dermatology Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,1,31]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/exd.14768"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-021-00654-z"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.14386"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.12070"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.14523"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/exd.14765"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18720"},{"key":"e_1_2_9_9_1","unstructured":"Ministry of Health Labour and Welfare.Table\u00a02\u201320 Number of persons with specific (intractable) disease healthcare certificate by disease.2020. Available from:https:\/\/www.mhlw.go.jp\/english\/database\/db\u2010hh\/2\u20101.html. Accessed 10 Mar 2023."},{"key":"e_1_2_9_10_1","first-page":"3","article-title":"Rare diseases, orphan drugs, and their regulation in Asia: current status and future perspectives","volume":"1","author":"Song P","year":"2012","journal-title":"Intractable Rare Dis Res"},{"key":"e_1_2_9_11_1","first-page":"45","article-title":"Research and development on intractable & rare diseases in Japan: contribution of the National Institute of Public Health to research program management","volume":"68","author":"Takemura S","year":"2019","journal-title":"J Natl Inst Public Health"},{"key":"e_1_2_9_12_1","unstructured":"SeikansenN.Pustular psoriasis (generalized) (designated intractable disease 37).2020. Available from:https:\/\/www.nanbyou.or.jp\/entry\/168. Accessed 11 Mar 2023."},{"key":"e_1_2_9_13_1","unstructured":"Japan Intractable Diseases Information Center.New countermeasures against intractable diseases started in 2015.2015. Available from:https:\/\/www.nanbyou.or.jp\/entry\/4141. Accessed 10 Mar 2023."},{"key":"e_1_2_9_14_1","unstructured":"Ministry of Health Labour and Welfare.Designated intractable diseases enforced on November 1 2021 (notification numbers 288 334 to 338).2021. Available from:https:\/\/www.mhlw.go.jp\/stf\/newpage_21649.html. Accessed 10 Mar 2023."},{"key":"e_1_2_9_15_1","unstructured":"Japan Intractable Diseases Information Center.What is an intractable disease?Available from:https:\/\/www.nanbyou.or.jp\/entry\/5460. Accessed 4 Jan 2023."},{"key":"e_1_2_9_16_1","unstructured":"Statistics Bureau of Japan.The Statistical Handbook of Japan 2022.2022. Available from:https:\/\/www.stat.go.jp\/english\/data\/handbook\/index.html. Accessed 10 Mar 2023."},{"key":"e_1_2_9_17_1","unstructured":"Japan Intractable Diseases Information Center.Number of recipients of specified medical expenses (designated intractable diseases).2020. Available from:https:\/\/www.stat.go.jp\/english\/data\/handbook\/pdf\/2022all.pdf. Accessed 12 Mar 2023."},{"key":"e_1_2_9_18_1","unstructured":"Ministry of Health Labour and Welfare.Physician Dentist and Pharmacist Statistics (formerly Physician Dentist and Pharmacist Survey).2020. Available from:https:\/\/www.mhlw.go.jp\/toukei\/list\/33\u201020.html. Accessed 10 Mar 2023."},{"key":"e_1_2_9_19_1","unstructured":"Statistics Bureau of Japan.Basic Resident Register Population Movement Report 2020 (Raiwa 2) Results.2020. Available from:https:\/\/www.stat.go.jp\/data\/idou\/2020np\/jissu\/youyaku\/index.html. Accessed 11 Mar 2023."},{"key":"e_1_2_9_20_1","unstructured":"Statistics Bureau of Japan.Basic Resident Register Population Movement Report.2020. Available from:https:\/\/www.stat.go.jp\/data\/idou\/index.html. Accessed 12 Mar 2023."},{"key":"e_1_2_9_21_1","unstructured":"Research Group on Rare and Intractable Skin Diseases.Research on rare and intractable skin diseases.2023. Last updated: April 20 2023. Available from:https:\/\/kinan.info\/. Acessed 9 May 2023."},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/dth.14136"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16362"},{"key":"e_1_2_9_24_1","unstructured":"OguroK MoriS NagamoriS NishimuraY MizutaniK ItoT.Overview survey on measures aganist intractable diseases in the government (before the enforcement of the Intractable Diseases Law).2015Available from:https:\/\/nanbyo.jp\/news2\/150207nisizawahan\/oguro.pdf. Accessed March 12 2023."},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.4091\/iken.29.027"},{"key":"e_1_2_9_26_1","first-page":"367","article-title":"The distribution of hospital nurses and associated factors","volume":"63","author":"Sakata Y","year":"2016","journal-title":"Nihon Koshu Eisei Zasshi"},{"key":"e_1_2_9_27_1","unstructured":"Ministy of Health Labour and Welfare.Table\u00a02\u201327 Number of beds and beds per 100 000 population by type of institution and prefecture.2022. Available from:https:\/\/www.mhlw.go.jp\/english\/database\/db\u2010hh\/2\u20102.html. Accessed 11 Mar 2023."},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2018.06.038"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes14061297"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2022.2116003"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.2307\/2574222"},{"key":"e_1_2_9_33_1","unstructured":"Information Center for Specific Pediatric Chronic Diseases.The registration situation to database such as children chronic specific disease children. Available from:https:\/\/www.shouman.jp\/research\/totalization. Accessed 10 Mar 2023."}],"container-title":["The Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1346-8138.17089","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T05:21:51Z","timestamp":1712035311000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1346-8138.17089"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,31]]},"references-count":32,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["10.1111\/1346-8138.17089"],"URL":"https:\/\/doi.org\/10.1111\/1346-8138.17089","archive":["Portico"],"relation":{},"ISSN":["0385-2407","1346-8138"],"issn-type":[{"value":"0385-2407","type":"print"},{"value":"1346-8138","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,31]]},"assertion":[{"value":"2023-08-29","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-12-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-31","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}