{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:05:07Z","timestamp":1791227107876,"version":"4.1.0"},"reference-count":57,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,12,28]],"date-time":"2018-12-28T00:00:00Z","timestamp":1545955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Photochem &amp; Photobiology"],"published-print":{"date-parts":[[2019,1]]},"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 caused by deficiency in repair of <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> lesions generated by ultraviolet radiation and other compounds. Patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> display pigmentary change and numerous skin cancers in sun\u2010exposed sites, and some patients show exaggerated severe sunburns even upon minimum sun exposure as well as neurological symptoms. We conducted a nationwide survey for <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> since 1980. In Japan, the frequency of the <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> complementation group A is the highest, followed by the variant type; while in the Western countries, those of groups C or D are the highest. Regarding skin cancers in <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>, basal cell carcinoma was the most frequent cancer that afflicted patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>, 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> has decreased in these 20 years, and the age of onset of developing skin cancers is higher than those previously observed, owing to early diagnosis and education to patients and care takers on strict prevention from sunlight for patients with <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>. On the other hand, the effective therapy for neurological <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> has not been established yet, and this needs to be done urgently.<\/jats:p>","DOI":"10.1111\/php.13052","type":"journal-article","created":{"date-parts":[[2018,12,19]],"date-time":"2018-12-19T14:35:15Z","timestamp":1545230115000},"page":"140-153","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Characteristics of Xeroderma Pigmentosum in Japan: Lessons From Two Clinical Surveys and Measures for Patient Care"],"prefix":"10.1111","volume":"95","author":[{"given":"Chikako","family":"Nishigori","sequence":"first","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eiji","family":"Nakano","sequence":"additional","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":"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":"Mariko","family":"Tsujimoto","sequence":"additional","affiliation":[{"name":"Department of Dermatology Graduate School of Medicine Kobe University Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takehiro","family":"Ueda","sequence":"additional","affiliation":[{"name":"Division of Neurology Graduate School of Medicine Kobe University Kobe Hyogo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,12,28]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/218652a0"},{"key":"e_1_2_14_3_1","first-page":"3837","article-title":"The human DNA repair gene, ERCC2 (XPD), corrects ultraviolet hypersensitivity and ultraviolet hypermutability of a shuttle vector replicated in xeroderma pigmentosum group D cells","volume":"54","author":"G\u00f6z\u00fckara E. 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