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CDS may present with skin changes, most commonly congenital non\u2010 bullous ichthyosiform erythroderma, however erythrokeratoderma\u2010like findings have been rarely reported in CDS patients. Herein, we report clinical, histopathological and genetic findings of four patients with CDS presenting with different clinical forms of erythrokeratoderma (three with progressive symmetric erythrokeratoderma\u2010like features and one with erythrokeratoderma variabilis (EKV)\u2010like features), including one patient with a novel mutation in <jats:italic>ABHD5<\/jats:italic>. Although the typical skin finding of CDS syndrome is reported as non\u2010bullous congenital ichthyosiform erythroderma, CDS should also be in the differential diagnosis in patients with EKV\u2010like lesions.<\/jats:p>","DOI":"10.1111\/pde.15654","type":"journal-article","created":{"date-parts":[[2024,6,18]],"date-time":"2024-06-18T02:25:24Z","timestamp":1718677524000},"page":"1174-1178","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Four cases of <scp>Chanarin\u2010Dorfman<\/scp> syndrome presenting with different types of erythrokeratoderma"],"prefix":"10.1111","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2847-9127","authenticated-orcid":false,"given":"Tubanur","family":"\u00c7etinarslan","sequence":"first","affiliation":[{"name":"Department of Dermatology and Venereology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Havva","family":"Yaz\u0131c\u0131","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Metabolism and Nutrition Ege University  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kadri Murat","family":"Erdo\u011fan","sequence":"additional","affiliation":[{"name":"Genetic Diagnosis Center Tepecik Training and Research Hospital, University of Health Sciences  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sema","family":"Kalkan U\u00e7ar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Metabolism and Nutrition Ege University  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00f6ksu","family":"Dalg\u0131\u00e7","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gizem","family":"Kaya","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esra","family":"Er","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Metabolism and Nutrition Dr. Beh\u00e7et Uz Pediatric Diseases and Surgery Training and Research Hospital  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cemal","family":"Bila\u00e7","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peyker","family":"Temiz","sequence":"additional","affiliation":[{"name":"Pathology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7720-5491","authenticated-orcid":false,"given":"Aylin","family":"T\u00fcrel Ermertcan","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Manisa Celal Bayar University  Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Regina","family":"F\u00f6lster\u2010Holst","sequence":"additional","affiliation":[{"name":"Department of Dermatology\u2010Venereology and Allergology Universit\u00e4tsklinikum Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,6,17]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2022.03.016"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.4274\/tjh.93798"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14794"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2005.06828.x"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2011.01429.x"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12944-019-1181-6"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.4103\/ijd.ijd_1154_20"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2009.10.016"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0646-9"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.29252\/.22.6.415"},{"issue":"665","key":"e_1_2_10_12_1","first-page":"668","article-title":"Generalized erythematous plaques. 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