{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T17:49:40Z","timestamp":1789148980901,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,12,20]],"date-time":"2023-12-20T00:00:00Z","timestamp":1703030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Refractory pruritus is the most distressing, disease\u2010related symptom in patients with dystrophic epidermolysis bullosa (DEB), inducing an itch\u2010scratch\u2010blister cycle. Chronic inflammation is a hallmark of DEB, thus upregulation of inflammatory cytokines and Janus kinase (JAK) signaling may play a role in DEB\u2010related pruritus. We retrospectively reviewed the medical records of DEB patients with refractory pruritus who were treated with either baricitinib, a JAK1\/2 inhibitor, or upadacitinib, a selective JAK1 inhibitor. Patients received baricitinib (4\u2009mg) or upadacitinib (15\u2009mg) once a day for 2\u201332\u2009weeks. A total of 12 DEB patients (six recessive DEB and six dominant DEB) were included in this study. The mean\u00b1SD baseline pruritus visual analog scale (VAS) score was 7.5\u2009\u00b1\u20091.7. Upadacitinib or baricitinib treatment resulted in a rapid and sustained decrease in itch. Four out of 12 patients (33.3%) and seven out of 10 patients (70%) showed a decrease of at least 3 points in the pruritus VAS score from baseline at weeks 2 and 4, respectively. The mean percentage changes from baseline in pruritus VAS scores at weeks 2 and 4 were \u221242.9% and \u221252.7%, respectively. Subgroup analysis showed greater reductions in the pruritus VAS score in the baricitinib group (<jats:italic>n<\/jats:italic>\u2009=\u20095) compared to the upadacitinib group (<jats:italic>n<\/jats:italic>\u2009=\u20097), and in patients with epidermolysis bullosa pruriginosa (<jats:italic>n<\/jats:italic>\u2009=\u20093) compared to other subtypes of DEB (<jats:italic>n<\/jats:italic>\u2009=\u20099); however, these differences did not reach statistical significance. Three out of 10 (33.3%) patients showed at least a 2\u2010point reduction in pain intensity from baseline at week 4. Eight out of 12 patients (66.7%) also showed a reduction in the number of new blisters, which correlated with a reduction in the pruritus score. No patient discontinued treatment because of serious adverse events. Our results suggest that JAK1 or JAK1\/2 inhibitors could be a promising treatment option for DEB\u2010related pruritus. Long\u2010term safety should be assessed in future studies.<\/jats:p>","DOI":"10.1111\/1346-8138.17079","type":"journal-article","created":{"date-parts":[[2023,12,20]],"date-time":"2023-12-20T08:12:21Z","timestamp":1703059941000},"page":"441-447","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Efficacy of oral <scp>JAK1<\/scp> or <scp>JAK1<\/scp>\/2 inhibitor for treating refractory pruritus in dystrophic epidermolysis bullosa: A retrospective case series"],"prefix":"10.1111","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1314-6829","authenticated-orcid":false,"given":"Il Joo","family":"Kwon","sequence":"first","affiliation":[{"name":"Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute Yonsei University College of Medicine  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7369-7310","authenticated-orcid":false,"given":"Song\u2010Ee","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute Yonsei University College of Medicine  Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2327-4755","authenticated-orcid":false,"given":"Soo\u2010Chan","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Yongin Severance Hospital, Cutaneous Biology Research Institute Yonsei University College of Medicine  Yongin Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4720-9955","authenticated-orcid":false,"given":"Sang Eun","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Gangnam Severance 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