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In 1994 a new classification system for PXE was published as the result of a consensus conference. Since then the <jats:italic>ABCC6<\/jats:italic> gene has been discovered. We propose that there is a need for a classification system incorporating all relevant systemic symptoms and signs, based on standardized clinical, histological, and molecular biological examination techniques. We re\u2010evaluated the histopathologic PXE signs and propose a classification system with unambiguous criteria leading to a consistent diagnosis of definitive, probable, or possible PXE world\u2010wide. We put this proposed classification forward to encourage further debate on the diagnosis of this multi\u2010organ disorder. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.33329","type":"journal-article","created":{"date-parts":[[2010,3,26]],"date-time":"2010-03-26T18:22:02Z","timestamp":1269627722000},"page":"1049-1058","source":"Crossref","is-referenced-by-count":155,"title":["Proposal for updating the pseudoxanthoma elasticum classification system and a review of the clinical findings"],"prefix":"10.1002","volume":"152A","author":[{"given":"Astrid S.","family":"Plomp","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"Toonstra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur A.B.","family":"Bergen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marijke R.","family":"van Dijk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paulus T.V.M.","family":"de Jong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,3,26]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1673.1991.tb03000.x"},{"key":"e_1_2_9_3_1","first-page":"1112","article-title":"Identification of heterozygote carriers in families with a recessive form of pseudoxanthoma elasticum (PXE)","volume":"12","author":"Bacchelli B","year":"1999","journal-title":"Mod Pathol"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1997.18711957.x"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2003.173"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2372041136"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jid.5700129"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/76109"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2007.08076.x"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s001090100275"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1159\/000251388"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2004.030171"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9509"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2006.07286.x"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0039-6257(82)90158-8"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02924627"},{"key":"e_1_2_9_17_1","first-page":"1","article-title":"Morphological changes in pseudoxanthoma elasticum and senile skin","volume":"83","author":"Danielsen L","year":"1979","journal-title":"Acta Derm Venereol Suppl (Stockh)"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2008.12.006"},{"key":"e_1_2_9_19_1","first-page":"118","volume-title":"Angioid streaks and associated diseases. 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