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A high index of suspicion and recognition of the characteristic histological findings frequently facilitate a correct diagnosis, but the distribution of the gastrointestinal pathology and its microscopic features may be atypical. The pathologist thus requires a thorough knowledge of both the typical and many atypical faces of this disease, for which various documented therapies often still prove ineffective. Close correlation with clinical findings, including any pertinent ectodermal abnormalities, and careful examination of biopsies derived from polypoid and endoscopically spared mucosa will ensure a timely and correct diagnosis in patients with this enigmatic syndrome.<\/jats:p>","DOI":"10.1136\/jclinpath-2014-202488","type":"journal-article","created":{"date-parts":[[2014,7,9]],"date-time":"2014-07-09T00:23:02Z","timestamp":1404865382000},"page":"891-897","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":67,"title":["Cronkhite\u2013Canada syndrome six decades on: the many faces of an enigmatic disease"],"prefix":"10.1136","volume":"67","author":[{"given":"Tomas","family":"Slavik","sequence":"first","affiliation":[{"name":"Ampath Pathology Laboratories, Pretoria, South Africa"},{"name":"Department of Anatomical Pathology, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth Anne","family":"Montgomery","sequence":"additional","affiliation":[{"name":"Department of Pathology, Johns Hopkins Medical Institutions and School of Medicine, Baltimore, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2014,7,8]]},"reference":[{"key":"2025120521065172000_67.10.891.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195506162522401"},{"key":"2025120521065172000_67.10.891.2","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S0016-5085(66)80106-3","article-title":"Diffuse gastrointestinal polyposis with ectodermal changes. 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