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Treatment of three patients with the IL-1 antagonists anakinra and canakinumab resulted in rapid clinical improvement and reversal of the PRP-associated molecular signature with a 50% improvement in skin lesions after 2 to 3 weeks. This transcriptional signature was consistent with in vitro stimulation of keratinocytes with IL-1\u03b2. With the central role of IL-1\u03b2 underscoring its potential as a therapeutic target, our findings propose a redefinition of PRP as an autoinflammatory keratinization disorder. Further clinical trials are needed to validate the efficacy of IL-1\u03b2 antagonists in PRP.<\/jats:p>","DOI":"10.1126\/sciadv.ado2365","type":"journal-article","created":{"date-parts":[[2024,7,3]],"date-time":"2024-07-03T13:59:13Z","timestamp":1720015153000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":16,"title":["Targeting IL-1 controls refractory pityriasis rubra pilaris"],"prefix":"10.1126","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2123-8777","authenticated-orcid":true,"given":"Eloi","family":"Schmauch","sequence":"first","affiliation":[{"name":"Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA."},{"name":"Computer Science and Artificial Intelligence Laboratory, Massachusetts Institute of Technology, Cambridge, MA 02139, USA."},{"name":"A.I. 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