{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T03:51:36Z","timestamp":1788580296304,"version":"build-2803163510"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2024,3,13]],"date-time":"2024-03-13T00:00:00Z","timestamp":1710288000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Ministry of Science and Technology Council","award":["113-2811-B-006-002"],"award-info":[{"award-number":["113-2811-B-006-002"]}]},{"DOI":"10.13039\/501100004844","name":"National Cheng Kung University Hospital","doi-asserted-by":"publisher","award":["NCKUH-11102006"],"award-info":[{"award-number":["NCKUH-11102006"]}],"id":[{"id":"10.13039\/501100004844","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004844","name":"National Cheng Kung University Hospital","doi-asserted-by":"publisher","award":["NCKUH-11209004"],"award-info":[{"award-number":["NCKUH-11209004"]}],"id":[{"id":"10.13039\/501100004844","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,16]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Dominant dystrophic epidermolysis bullosa (DDEB) is characterized by trauma-induced blisters and, in some individuals, intense pruritus. Precisely what causes itch in DDEB and optimal ways to reduce it have not been fully determined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To characterize DDEB skin transcriptomes to identify therapeutic targets to reduce pruritus in patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Using bulk RNA sequencing, we evaluated affected and unaffected skin biopsy samples from six patients with DDEB (all with the very itchy pruriginosa subtype) and four healthy individuals. Single-cell transcriptomes of affected (n = 2) and unaffected (n = 1) DDEB skin and healthy skin (n = 2) were obtained. Dupilumab treatment was provided for three patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The skin bulk transcriptome showed significant enrichment of T helper (Th)1\/2 and Th17 pathways in affected DDEB skin compared with nonlesional DDEB skin and healthy skin. Single-cell transcriptomics showed an association of glycolytically active GATA3+ Th2 cells in affected DDEB skin. Treatment with dupilumab in three people with DDEB led to significantly reduced visual analogue scale (VAS) itch scores after 12 weeks (mean VAS 3.83) compared with pretreatment (mean VAS 7.83). Bulk RNAseq and quantitative polymerase chain reaction showed that healthy skin and dupilumab-treated epidermolysis bullosa (EB) pruriginosa skin have similar transcriptomic profiles and reduced Th1\/Th2 and Th17 pathway enrichment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Single-cell RNAseq helps define an enhanced DDEB-associated Th2 profile and rationalizes drug repurposing of anti-Th2 drugs in treating DDEB pruritus.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/bjd\/ljae110","type":"journal-article","created":{"date-parts":[[2024,3,12]],"date-time":"2024-03-12T02:32:44Z","timestamp":1710210764000},"page":"252-260","source":"Crossref","is-referenced-by-count":10,"title":["Dominant dystrophic epidermolysis bullosa is associated with glycolytically active\n                    <i>GATA3+<\/i>\n                    T helper 2 cells which may contribute to pruritus in lesional skin"],"prefix":"10.1093","volume":"191","author":[{"given":"Wilson Jr F","family":"Aala","sequence":"first","affiliation":[{"name":"Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ping-Chen","family":"Hou","sequence":"additional","affiliation":[{"name":"Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan,\u00a0Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi-Kai","family":"Hong","sequence":"additional","affiliation":[{"name":"Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan,\u00a0Taiwan"},{"name":"International Center for Wound Repair and Regeneration, National Cheng Kung University, Tainan, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Chen","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan,\u00a0Taiwan"},{"name":"International Center for Wound Repair and Regeneration, National Cheng Kung University, Tainan, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Rong","family":"Lee","sequence":"additional","affiliation":[{"name":"School of Medicine, College of Medicine, National Cheng Kung University , Tainan , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-Ting","family":"Tu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan,\u00a0Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marieta","family":"Papanikolaou","sequence":"additional","affiliation":[{"name":"St John\u2019s Institute of Dermatology, School of Basic and Medical Biosciences, King\u2019s College London , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natashia","family":"Benzian-Olsson","sequence":"additional","affiliation":[{"name":"St John\u2019s Institute of Dermatology, School of Basic and Medical Biosciences, King\u2019s College London , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandros","family":"Onoufriadis","sequence":"additional","affiliation":[{"name":"St John\u2019s Institute of Dermatology, School of Basic and Medical Biosciences, King\u2019s College London , London , UK"},{"name":"Laboratory of Medical Biology and Genetics, School of Medicine, Aristotle University of Thessaloniki , Thessaloniki , Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"I Chen Harn","sequence":"additional","affiliation":[{"name":"Department of Pathology, Keck School of Medicine, University of Southern California , Los Angeles, CA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daw-Yang","family":"Hwang","sequence":"additional","affiliation":[{"name":"National Institute of Cancer Research, National Health Research Institutes , Tainan , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siao Muk","family":"Cheng","sequence":"additional","affiliation":[{"name":"National Institute of Cancer Research, National Health Research Institutes , Tainan , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8297-433X","authenticated-orcid":false,"given":"Kurt","family":"Lu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Feinberg School of Medicine, 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