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Specific genodermatoses, including Netherton syndrome, epidermolysis bullosa pruriginosa, and hyper\u2010IgE syndrome, are Th2 skewed diseases with activation of type 2 inflammation. We performed this systematic review to investigate the therapeutic role of dupilumab in the treatment of genodermatosis. A systematic search was conducted of the PubMed, Embase, Web of Science, and Cochrane databases from inception to December 13, 2021. The review included studies with relevant terms including \u201cdupilumab,\u201d \u201cgenodermatosis\u201d, \u201cNetherton syndrome\u201d, \u201cichthyosis\u201d, \u201cepidermolysis bullosa\u201d and \u201chyper\u2010IgE syndrome\u201d. The initial search yielded 2,888 results, of which 28 studies and 37 patients with genodermatosis were enrolled. The assessed genodermatoses included Netherton syndrome, epidermolysis bullosa pruriginosa, hyper\u2010IgE syndrome, Hailey\u2010Hailey disease, and severe eczema associated with genetic disorders. Most of the reported cases showed significant clinical improvement after the initiation of dupilumab treatment without major adverse events. Decreased immunoglobulin\u00a0E levels and cytokine normalization have also been documented. In conclusion, Dupilumab may have a potential therapeutic role in certain genodermatoses skewed towards T\u00a0helper 2 (Th2) immunity, including Netherton syndrome, epidermolysis bullosa pruriginosa, hyper\u2010IgE syndrome, Hailey\u2010Hailey disease, and severe eczema associated with some genetic disorders.<\/jats:p>","DOI":"10.1111\/ddg.14924","type":"journal-article","created":{"date-parts":[[2023,1,19]],"date-time":"2023-01-19T15:19:16Z","timestamp":1674141556000},"page":"7-17","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Dupilumab in the treatment of genodermatosis: A systematic review"],"prefix":"10.1111","volume":"21","author":[{"given":"Po\u2010Chien","family":"Wu","sequence":"first","affiliation":[{"name":"Department of Dermatology Chang Gung Memorial Hospital  Linkou Taoyuan 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