{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T00:30:24Z","timestamp":1787617824367,"version":"build-2736575974"},"reference-count":27,"publisher":"Wiley","issue":"2-3","license":[{"start":{"date-parts":[[2014,2,19]],"date-time":"2014-02-19T00:00:00Z","timestamp":1392768000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Photoderm Photoimm Photomed"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Xeroderma pigmentosum (<jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>) is a rare autosomal recessive disease of deoxyribonucleic acid (<jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content>) repair with ultraviolet (<jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content>) radiation sensitivity and a 10\u2009000\u2010fold increased risk of skin cancer. Symptoms include: freckle\u2010like pigmentation in sun\u2010exposed skin before age 2 years, severe burns after minimal sun exposure (50% of patients) and damage to exposed surfaces of the eyes with loss of vision and ocular cancer. About 25% of patients develop a progressive neurodegeneration. The combination of an inherited inability to repair <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content>\u2010induced <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> damage and environmental exposure to <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content> must occur for cutaneous and ocular symptoms to develop. There is no cure for <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>, but many of its manifestations may be reduced or prevented through consistent <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content> protection; thus <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> serves as a model for sun protection of patients with marked photosenstivity. Sun protective clothing including hats, sunglasses and face shields, sun screen lotions and avoidance of environmental sources of <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content> are cornerstones of prevention of skin and eye damage and cancer. Although <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> is a serious disease with the potential for limitation of life expectancy, <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content> patients can live active lives while at the same time avoiding <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/phpp.12108","type":"journal-article","created":{"date-parts":[[2014,1,14]],"date-time":"2014-01-14T08:14:58Z","timestamp":1389687298000},"page":"146-152","source":"Crossref","is-referenced-by-count":62,"title":["Living with xeroderma pigmentosum: comprehensive photoprotection for highly photosensitive patients"],"prefix":"10.1111","volume":"30","author":[{"given":"Deborah","family":"Tamura","sequence":"first","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Institutes of Health National Cancer Institute  Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"DiGiovanna","sequence":"additional","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Institutes of Health National Cancer Institute  Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sikandar G.","family":"Khan","sequence":"additional","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Institutes of Health National Cancer Institute  Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth H.","family":"Kraemer","sequence":"additional","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Institutes of Health National Cancer Institute  Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,2,19]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.083022"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.426"},{"key":"e_1_2_11_4_1","first-page":"1654","volume-title":"Fitzpatrick's dermatology in general medicine","author":"Ruenger TM","year":"2012"},{"key":"e_1_2_11_5_1","first-page":"232","article-title":"Report of three sisters with XP\u2010E, a rare xeroderma pigmentosum complementation group","volume":"1","author":"Fischer E","year":"1984","journal-title":"Photodermatol"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2008.48"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1987.01660260111026"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0625.2008.00763.x"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-211-69500-5_51"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-4362.1999.00751.x"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2008.01.009"},{"key":"e_1_2_11_12_1","first-page":"319","article-title":"Xeroderma pigmentosum: a nursing perspective","volume":"2","author":"Tokar IP","year":"1990","journal-title":"Dermatol Nurs"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2008.06.030"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2011.03.001"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0039-6257(02)00400-9"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2006.12.020"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2012.12.044"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn2559"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.3.1335"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws317"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn126"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1186\/2051-5960-1-4"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2011.04.027"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.39485.698356.AD"},{"key":"e_1_2_11_25_1","first-page":"789","volume-title":"Treatment of skin disease","author":"Tamura D","year":"2010"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(97)70069-0"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2009.50"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2008.01.014"}],"container-title":["Photodermatology, Photoimmunology &amp; 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