{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T11:50:41Z","timestamp":1792410641493,"version":"4.4.0"},"reference-count":30,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Health Science Reports"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aim<\/jats:title>\n                    <jats:p>Maple Syrup Urine Disease (MSUD) is a metabolic disorder affecting branched\u2010chain amino acid metabolism. While neurological symptoms are well\u2010characterized, cutaneous manifestations such as acrodermatitis dysmetabolica (AD) caused by isoleucine deficiency remain underrecognized. This systematic review aimed to summarize reported cases of AD or acrodermatitis enteropathica\u2010like eruptions in patients with MSUD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic search of PubMed, Scopus, and Web of Science was conducted through June 2026. Case reports and studies describing dermatologic findings in MSUD patients with confirmed isoleucine deficiency were included.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>19 studies reporting 20 patients were investigated. Most cases involved infants presenting with erythematous, scaly, or erosive lesions primarily in periorificial and acral regions. All patients exhibited low isoleucine levels with normal zinc status. Isoleucine supplementation resulted in clinical improvement in nearly all cases.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Acrodermatitis dysmetabolica is a treatable dermatologic complication of MSUD, typically arising from dietary isoleucine deficiency. Early diagnosis and monitoring of amino acid levels are critical for effective management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hsr2.73230","type":"journal-article","created":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T04:57:42Z","timestamp":1790139462000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Acrodermatitis Dysmetabolica as a Cutaneous Manifestation of Isoleucine Deficiency in Maple Syrup Urine Disease: A Systematic Review of Reported Cases"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5762-5752","authenticated-orcid":false,"given":"Bahareh","family":"Abtahi\u2010naeini","sequence":"first","affiliation":[{"name":"Skin Diseases and Leishmaniasis Research Center Isfahan University of Medical Sciences Isfahan Isfahan Iran"},{"name":"Pediatric Dermatology Division of Department of Pediatrics, Imam Hossein Children's Hospital Isfahan University of Medical Sciences Isfahan Isfahan Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5543-5536","authenticated-orcid":false,"given":"Motahar","family":"Heidari\u2010Beni","sequence":"additional","affiliation":[{"name":"Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non\u2010Communicable Disease Isfahan University of Medical Sciences Isfahan Isfahan Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noushin","family":"Rostampour","sequence":"additional","affiliation":[{"name":"Metabolic Liver Disease Research Center Isfahan University of Medical Sciences Isfahan Isfahan Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3040-7256","authenticated-orcid":false,"given":"Naeimeh","family":"Davoudi","sequence":"additional","affiliation":[{"name":"Skin Diseases and Leishmaniasis Research Center Isfahan University of Medical Sciences Isfahan Isfahan Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1475-9344","authenticated-orcid":false,"given":"Sarah","family":"Seyedyousefi","sequence":"additional","affiliation":[{"name":"Skin Diseases and Leishmaniasis Research Center Isfahan University of Medical Sciences Isfahan Isfahan Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,22]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/plb.13771"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.2147\/TACG.S125962"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12876"},{"issue":"5","key":"e_1_2_14_5_1","first-page":"389","article-title":"[Isolated Isoleucine Deficiency in Diet Therapy of a Case of Maple Syrup Urine Disease]","volume":"19","author":"Sanjurjo P.","year":"1983","journal-title":"Anales Espanoles de Pediatria"},{"issue":"4","key":"e_1_2_14_6_1","first-page":"9750","article-title":"Acrodermatitis Dysmetabolica Secondary to Isoleucine Deficiency in Infant With Maple Syrup Urine Disease","volume":"15","author":"Alkhayal F. 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