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To reassess published hypomelanotic and hypermelanotic lesions that did not follow Blaschko lines nor a phylloid pattern of mosaicism, we carried out an extensive and critical review of the worldwide literature. Seventy\u2010four retrieved cases consisted of lateralized hypomelanotic lesions arranged in a flag\u2010like pattern or appearing as large patches of grossly oval or angulated shape and sharp, serrated margins. Sometimes lesions harboured maculopapular melanocytic naevi or cooccurred with other segmentally arranged naevi. A probably non\u2010random association with extracutaneous anomalies was also reported on rare occasions. In 70 cases, lateralized hypermelanotic patches were arranged in a flag\u2010like pattern that often appeared as large quadrangular patches. Sometimes lesions harboured Spitz naevi. Ten cases belonged to phacomatosis melanorosea, whereas several others were part of so far uncategorized cases of phacomatosis pigmentovascularis. Flag\u2010like hypomelanosis is a distinct naevus type, for which the term \u2018flag\u2010like hypomelanotic naevus\u2019 is suggested. Its cooccurrence with extracutaneous abnormalities might represent a specific syndrome. Flag\u2010like hypermelanosis is a distinct naevus type, for which the term \u2018flag\u2010like hypermelanotic naevus\u2019 is suggested. Its co\u2010occurrence with naevus roseus defines phacomatosis melanorosea. Flag\u2010like hypermelanotic naevus should be distinguished from the checkerboard\u2010like areas of darker skin as observed in chimaeras.<\/jats:p>","DOI":"10.1111\/jdv.13077","type":"journal-article","created":{"date-parts":[[2015,3,6]],"date-time":"2015-03-06T13:21:15Z","timestamp":1425648075000},"page":"2088-2099","source":"Crossref","is-referenced-by-count":14,"title":["Segmental hypomelanosis and hypermelanosis arranged in a checkerboard pattern are distinct naevi: flag\u2010like hypomelanotic naevus and flag\u2010like hypermelanotic naevus"],"prefix":"10.1111","volume":"29","author":[{"given":"D.","family":"Torchia","sequence":"first","affiliation":[{"name":"Department of Dermatology Queen Elizabeth Hospital King's Lynn UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Happle","sequence":"additional","affiliation":[{"name":"Department of Dermatology Freiburg University Medical Center Freiburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,3,6]]},"reference":[{"key":"e_1_2_5_2_1","first-page":"183.","volume-title":"Handbuch der Haut krankheiten","author":"Lesser E","year":"1884"},{"key":"e_1_2_5_3_1","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-642-38765-4","volume-title":"Mosaicism in Human Skin. 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