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The review will focus on the most common forms where dental manifestations can be crucial for a differential diagnosis of ED among individuals with hypodontia and oligodontia, and preferably where the investigations included persons who had a genetically verified diagnosis. Disturbances in tooth development are common and can appear as tooth agenesis, variations in size and shape of teeth, defects in the mineralized tissues, and problems in tooth eruption. Abnormalities in number, size, and shape of teeth, and reduced salivary secretion, present in isolated oligodontia as well as in hypohidrotic ED and incontinentia pigmenti. In some more rare EDs these symptoms appear in combination with clefts of lip and\/or palate in some affected individuals. Leukokeratosis in the oral mucosa presents in 70% of genetically confirmed cases of pachyonychia congenita. Also, orofacial function is often affected in ED, due to malformations, an incomplete dentition, and low salivary secretion which can compromise chewing, swallowing, and speech. In conclusion, there is a clinical overlap in oral signs and symptoms between isolated oligodontia and the most common EDs. Studies with genetically confirmed diagnoses and larger cohorts, as well as multicenter collaboration and the establishing of international registries, would create a basis for refined diagnostics, where oral examinations should be an integrated part of clinical assessment. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajmg.a.36571","type":"journal-article","created":{"date-parts":[[2014,4,9]],"date-time":"2014-04-09T21:32:23Z","timestamp":1397079143000},"page":"2465-2471","source":"Crossref","is-referenced-by-count":53,"title":["Orodental manifestations in ectodermal dysplasia\u2014A review"],"prefix":"10.1002","volume":"164","author":[{"given":"Birgitta","family":"Bergendal","sequence":"first","affiliation":[{"name":"National Oral Disability Centre for Rare Disorders The Institute for Postgraduate Dental Education J\u00f6nk\u00f6ping Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,4,9]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36243"},{"key":"e_1_2_9_3_1","first-page":"75","article-title":"Development and evaluation of a comprehensive screening for orofacial dysfunction","volume":"31","author":"Bakke M","year":"2007","journal-title":"Swed Dent J"},{"key":"e_1_2_9_4_1","article-title":"Oligodontia and ectodermal dysplasia\u2013on signs, symptoms, genetics, and outcomes of dental treatment","volume":"34","author":"Bergendal B","year":"2010","journal-title":"Swed Dent J"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-263X.2006.00741.x"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/9780813804644.ch3"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/00016350903160571"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.34045"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.06.001"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0544.2002.01159.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9969(84)90163-8"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2010.01376.x"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21384"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.arcped.2012.07.002"},{"key":"e_1_2_9_15_1","volume-title":"Dorland's illustrated medical dictionary","author":"Dorland WAN","year":"2007"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2011.12.009"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(92)70179-J"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1159\/000152419"},{"key":"e_1_2_9_19_1","first-page":"3","article-title":"Ectodermal dysplasias\u2013some recollections and a classification","volume":"24","author":"Freire\u2010Maia N","year":"1988","journal-title":"Birth Defects Orig Artic Ser"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prosdent.2005.03.013"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2842.1980.tb00447.x"},{"key":"e_1_2_9_22_1","first-page":"1345","article-title":"Incontinentia pigmenti. 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