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We report on the second instance of monozygotic twin discordance for PPV. Identical twins were observed shortly after birth, one affected by PPV and the other healthy, except for a standard Mongolian spot. Membrane examination was compatible with a monochorionic diamniotic pregnancy, and microsatellite analysis demonstrated monozygosity. This report confirms that PPV likely originates from a post\u2010zygotic mutation rising shortly after conception and affecting different cell lineages. Speculations about mechanisms linked to phenotypic discrepancies among identical twins were also put forward. \u00a9 2011 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.34140","type":"journal-article","created":{"date-parts":[[2011,8,4]],"date-time":"2011-08-04T00:12:30Z","timestamp":1312416750000},"page":"2253-2256","source":"Crossref","is-referenced-by-count":8,"title":["Monozygotic twin discordance for phacomatosis cesioflammea further supports the post\u2010zygotic mutation hypothesis"],"prefix":"10.1002","volume":"155","author":[{"given":"Marco","family":"Castori","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Sarazani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Binni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca 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