{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T23:29:21Z","timestamp":1782602961333,"version":"3.54.5"},"reference-count":31,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,11,23]],"date-time":"2022-11-23T00:00:00Z","timestamp":1669161600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100005565","name":"Janssen Biotech","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005565","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Netherton syndrome (NS) is a rare disorder of cornification associated with high morbidity. It is caused by bi\u2010allelic mutations in <jats:italic>SPINK5<\/jats:italic> encoding the serine protease inhibitor LEKTI. Previous studies have shown Th17 skewing with IL\u201023 upregulation in NS, raising the possibility that targeting these inflammatory pathways may alleviate disease manifestations. We ascertained the therapeutic efficacy of six doses of ustekinumab administered to three patients with NS over a period of 13\u2009months using the Ichthyosis Area and Severity Index (IASI), the Dermatology Life Quality Index (DLQI), a visual analogue scale (VAS) for itch and the peak\u2010pruritus numeric rating scale (PP\u2010NRS). Histopathology analysis including CD3, CD4, CD8 and interleukin 17 (IL\u201017) immunostaining, was performed at baseline and 4\u2009weeks following the last ustekinumab dose. Total IASI scores were reduced by 28% in two patients at week 16 with sustained response by week 56. No consistent improvement in DLQI, VAS for itch and PP\u2010NRS scores was observed. The inflammatory infiltrate and the degree of acanthosis were slightly reduced at week 56 as compared to baseline. No significant change in immunostaining of the various inflammatory markers was observed at week 56. In conclusion, this case series did not demonstrate a significant therapeutic effect of ustekinumab in NS.<\/jats:p>","DOI":"10.1111\/1346-8138.16645","type":"journal-article","created":{"date-parts":[[2022,11,24]],"date-time":"2022-11-24T13:19:09Z","timestamp":1669295949000},"page":"494-499","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Ustekinumab therapy for Netherton syndrome"],"prefix":"10.1111","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5387-5490","authenticated-orcid":false,"given":"Liat","family":"Samuelov","sequence":"first","affiliation":[{"name":"Division of Dermatology Tel Aviv Sourasky Medical Center  Tel Aviv Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University  Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Waseem","family":"Shehadeh","sequence":"additional","affiliation":[{"name":"Division of Dermatology Tel Aviv Sourasky Medical 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