{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T16:07:49Z","timestamp":1791389269505,"version":"4.3.3"},"reference-count":7,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,11,15]],"date-time":"2024-11-15T00:00:00Z","timestamp":1731628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,11,15]],"date-time":"2024-11-15T00:00:00Z","timestamp":1731628800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Klinikum der Universit\u00e4t M\u00fcnchen"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Clin Immunol"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Netherton syndrome (NS) is a rare inborn error of immunity (IEI) with an incidence of approximately 1:200,000 and the phenotypic triad of trichorrhexis invaginate (bamboo hair), congenital ichthyosiform erythroderma, and multiple atopic manifestations. Treatment options especially in infants are scarce and generally not licensed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Case report of a 9-week-old infant with NS treated with dupilumab off-label.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We report rapid and sustained resolution of allergic inflammation, deep symptom control including normalization of the skin microbiome, and catch-up somatic and psychomotor development without adverse drug reactions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Due to the high complication rate of NS, especially in the first years of life, we recommend treatment with dupilumab off-label immediately after the diagnosis has been established.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10875-024-01837-z","type":"journal-article","created":{"date-parts":[[2024,11,15]],"date-time":"2024-11-15T07:22:48Z","timestamp":1731655368000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Dupilumab in a 9-week-old with Netherton Syndrome Leads to Deep Symptom Control"],"prefix":"10.1007","volume":"45","author":[{"given":"Yannik","family":"Vollmuth","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narjes Abdulhameed","family":"Alelq","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franziska","family":"Sattler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabian","family":"Hauck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,11,15]]},"reference":[{"issue":"7","key":"1837_CR1","doi-asserted-by":"publisher","first-page":"1508","DOI":"10.1007\/s10875-022-01352-z","volume":"42","author":"A Bousfiha","year":"2022","unstructured":"Bousfiha A, et al. The 2022 update of IUIS Phenotypical classification for human inborn errors of immunity. J Clin Immunol 2022;42(7):1508\u201320.","journal-title":"J Clin Immunol"},{"issue":"11","key":"1837_CR2","doi-asserted-by":"publisher","first-page":"455","DOI":"10.1080\/21678707.2020.1857724","volume":"8","author":"E Petrova","year":"2020","unstructured":"Petrova E, Hovnanian A. Advances in understanding of Netherton syndrome and therapeutic implications. Expert Opin Orphan Drugs 2020;8(11):455\u201387.","journal-title":"Expert Opin Orphan Drugs"},{"issue":"2","key":"1837_CR3","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1007\/s00441-013-1558-1","volume":"351","author":"A Hovnanian","year":"2013","unstructured":"Hovnanian A. Netherton syndrome: skin inflammation and allergy by loss of protease inhibition. Cell Tissue Res 2013;351(2):289\u2013300.","journal-title":"Cell Tissue Res"},{"key":"1837_CR4","unstructured":"Zambruno G. Orphanet: Netherton syndrome 2008. [Cited 31 March 2024]. https:\/\/www.orpha.net\/en\/disease\/detail\/634"},{"issue":"4","key":"1837_CR5","doi-asserted-by":"publisher","first-page":"634","DOI":"10.3390\/biom13040634","volume":"13","author":"H Olbrich","year":"2023","unstructured":"Olbrich H, et al. Dupilumab in inflammatory skin diseases: a systematic review. Biomolecules 2023;13(4):634.","journal-title":"Biomolecules"},{"issue":"4","key":"1837_CR6","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1001\/jamadermatol.2021.5917","volume":"158","author":"Q Sun","year":"2022","unstructured":"Sun Q, et al. Development and initial validation of a Novel System to assess ichthyosis severity. JAMA Dermatol 2022;158(4):359\u201365.","journal-title":"JAMA Dermatol"},{"issue":"1","key":"1837_CR7","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1001\/archdermatol.2009.326","volume":"146","author":"AC Yan","year":"2010","unstructured":"Yan AC, et al. The safety and efficacy of pimecrolimus, 1%, cream for the treatment of Netherton syndrome: results from an exploratory study. Arch Dermatol 2010;146(1):57\u201362.","journal-title":"Arch Dermatol"}],"container-title":["Journal of Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-024-01837-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10875-024-01837-z","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-024-01837-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T15:46:04Z","timestamp":1777304764000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10875-024-01837-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,15]]},"references-count":7,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["1837"],"URL":"https:\/\/doi.org\/10.1007\/s10875-024-01837-z","relation":{},"ISSN":["0271-9142","1573-2592"],"issn-type":[{"value":"0271-9142","type":"print"},{"value":"1573-2592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,15]]},"assertion":[{"value":"17 July 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 November 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 November 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors have no conflicts of interest to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"42"}}