{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T00:28:33Z","timestamp":1787617713135,"version":"build-2736575974"},"reference-count":13,"publisher":"Wiley","issue":"S3","license":[{"start":{"date-parts":[[2016,8,18]],"date-time":"2016-08-18T00:00:00Z","timestamp":1471478400000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Health and Labor Sciences Research Grants, Research on intractable diseases"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2016,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Xeroderma pigmentosum (<jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>) is a rare autosomal recessive hereditary disease. Patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> have severe hypersensitivity to sunlight, resulting in skin cancers, and some patients have neurological symptoms. In Japan, <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> complementation group A (<jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\u2010A) is the most common form, and it is associated with severe neurological symptoms. We performed a nationwide survey on <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> to determine the present status of <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> in Japan. The distribution of complementation groups in Japan was considerably different from that in other countries, but there was a higher frequency in group A and the variant type, which is similar to previous reports in Japan. Basal cell carcinoma was the most frequent skin cancer that patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> developed, followed by squamous cell carcinoma and malignant melanoma. The frequency of these skin cancers in patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\u2010A has decreased, and these skin cancers have been occurring in much older people than those previously observed. Diagnosing <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> in patients at younger ages seems to encourage patients and their parents to use sun protection, which helps prevent skin cancer. We also created a tentative scale for classifying the severity of <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>, and we evaluated the neurological symptoms of <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\u2010A using this severity scale. Our classification correlated well with patients' age, suggesting that it may be useful and feasible in clinical practice to assess the progression of symptoms of each patient with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> and evaluate the effects of treatment in the future.<\/jats:p>","DOI":"10.1111\/exd.13082","type":"journal-article","created":{"date-parts":[[2016,8,19]],"date-time":"2016-08-19T02:40:26Z","timestamp":1471574426000},"page":"28-33","source":"Crossref","is-referenced-by-count":22,"title":["The present status of xeroderma pigmentosum in Japan and a tentative severity classification scale"],"prefix":"10.1111","volume":"25","author":[{"given":"Eiji","family":"Nakano","sequence":"first","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taro","family":"Masaki","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumio","family":"Kanda","sequence":"additional","affiliation":[{"name":"Division of Neurology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryusuke","family":"Ono","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seiji","family":"Takeuchi","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichi","family":"Moriwaki","sequence":"additional","affiliation":[{"name":"Department of Dermatology Osaka Medical College  Takatsuki Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chikako","family":"Nishigori","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University  Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,8,18]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.426"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jid.5700759"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1039\/B507471A"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrfmmm.2006.06.010"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(1999)14:1<9::AID-HUMU2>3.0.CO;2-6"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2008.01.014"},{"key":"e_1_2_7_8_1","first-page":"1135","article-title":"Genetics and skin cancer of xeroderma pigmentosum in Japan","volume":"78","author":"Takebe H","year":"1987","journal-title":"Jpn J Cancer Res"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1994.01690020057009"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.22"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.083022"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/carcin\/5.4.511"},{"key":"e_1_2_7_13_1","first-page":"113","article-title":"Xeroderma pigmentosum: clinical aspects","volume":"35","author":"Sato Y","year":"1988","journal-title":"Gann Monogr Cancer Res"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-0-387-09599-8_13"}],"container-title":["Experimental Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fexd.13082","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fexd.13082","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/exd.13082","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T01:11:14Z","timestamp":1694481074000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/exd.13082"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,8]]},"references-count":13,"journal-issue":{"issue":"S3","published-print":{"date-parts":[[2016,8]]}},"alternative-id":["10.1111\/exd.13082"],"URL":"https:\/\/doi.org\/10.1111\/exd.13082","archive":["Portico"],"relation":{},"ISSN":["0906-6705","1600-0625"],"issn-type":[{"value":"0906-6705","type":"print"},{"value":"1600-0625","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,8]]}}}