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Hereditary disorders associated with defective <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> repair are valuable models for understanding mechanisms that lead to hypo\u2010 and hyperpigmentation. Owing to the <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content>\u2010associated nature of abnormal pigmentary manifestations, the outcome of the activated <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> damage response (<jats:styled-content style=\"fixed-case\">DDR<\/jats:styled-content>) network could be the effector signal for alterations in pigmentation, ultimately manifesting as pigmentary abnormalities in repair\u2010deficient disorders. In this review, the role of the <jats:styled-content style=\"fixed-case\">DDR<\/jats:styled-content> network in the manifestation of pigmentary abnormalities in <jats:styled-content style=\"fixed-case\">NER<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">DSB<\/jats:styled-content> disorders is discussed with a special emphasis on <jats:styled-content style=\"fixed-case\">NER<\/jats:styled-content> disorders.<\/jats:p>","DOI":"10.1111\/pcmr.12720","type":"journal-article","created":{"date-parts":[[2018,6,25]],"date-time":"2018-06-25T11:18:26Z","timestamp":1529925506000},"page":"25-40","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Pigmentation abnormalities in nucleotide excision repair disorders: Evidence and hypotheses"],"prefix":"10.1111","volume":"32","author":[{"given":"Zeinab","family":"Kasraian","sequence":"first","affiliation":[{"name":"NAOS  Aix en Provence France"},{"name":"Univ. 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