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Skin involvement in HLCSD is typically described as scaly, erythrodermic, seborrhea\u2010like, or ichthyosiform, but there is a paucity of reports. We report three patients, including two siblings, with HLCSD and significant cutaneous manifestations including ichthyosiform dermatitis and a presentation with features of annular pustular psoriasis. In this report, we show that persistent, unexplained rash, even in the absence of other clinical findings, should warrant consideration and potential workup for HLCSD.<\/jats:p>","DOI":"10.1111\/pde.14586","type":"journal-article","created":{"date-parts":[[2021,4,19]],"date-time":"2021-04-19T08:03:22Z","timestamp":1618819402000},"page":"655-658","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Delayed diagnosis of holocarboxylase synthetase deficiency in three patients with prominent skin findings"],"prefix":"10.1111","volume":"38","author":[{"given":"Maxime","family":"Cadieux\u2010Dion","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Children\u2019s Mercy Hospital  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"Gannon","sequence":"additional","affiliation":[{"name":"Division of Clinical Genetics Department of Pediatrics Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brandon","family":"Newell","sequence":"additional","affiliation":[{"name":"Division of Dermatology Department of Pediatrics Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amy Jo","family":"Nopper","sequence":"additional","affiliation":[{"name":"Division of Dermatology Department of Pediatrics Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Janda","family":"Jenkins","sequence":"additional","affiliation":[{"name":"Division of Clinical Genetics Department of Pediatrics Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bryce","family":"Heese","sequence":"additional","affiliation":[{"name":"Division of Clinical Genetics Department of Pediatrics Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7407-7735","authenticated-orcid":false,"given":"Carol","family":"Saunders","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Children\u2019s Mercy Hospital  Kansas City MO USA"},{"name":"University of Missouri\u2010Kansas City School of Medicine  Kansas City MO USA"},{"name":"Center for Pediatric Genomic Medicine Children\u2019s Mercy Hospital  Kansas City MO USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,4,19]]},"reference":[{"key":"e_1_2_5_2_1","first-page":"1","article-title":"Severe neonatal holocarboxylase synthetase deficiency in west african siblings","volume":"20","author":"De Castro M","year":"2015","journal-title":"JIMD Rep"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2009.01357.x"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110402"},{"volume-title":"GeneReviews((R))","author":"Wolf B","key":"e_1_2_5_5_1"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2016.03.007"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3004041"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2008.09.006"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2006.00200.x"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2013.08.062"},{"issue":"4","key":"e_1_2_5_11_1","first-page":"267","article-title":"Inborn errors of biotin metabolism. 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