{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T17:26:30Z","timestamp":1786987590724,"version":"build-2736575974"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T00:00:00Z","timestamp":1776470400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,8,17]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Generalized pustular psoriasis (GPP) is a rare, life-threatening skin disease. Spesolimab, an anti-interleukin (IL)-36R monoclonal antibody, has shown efficacy in clinical trials, yet real-world data remain limited. We evaluated the real-world effectiveness and safety of spesolimab in patients with GPP and the influence of IL36RN mutation status on treatment response. A prospective study was conducted including patients with GPP treated with a single 900-mg intravenous infusion of spesolimab, with follow-up at 1 week, 1\u2005month, 3\u2005months and 6\u2005months. Disease severity was assessed using the Generalized Pustular Psoriasis Area and Severity Index (GPPASI) score. Screening for the IL36RN c.80T&amp;gt;C(p.L27P) mutation was performed using Sanger sequencing. In total, 14 patients (9 women, 5 men; mean age 39.1\u2005years) were included. The mean baseline GPPASI score was 3.61, which rapidly decreased to 1.3 at day 7, 0.52 at 1\u2005month and 0.29 at 3\u2005months, yet slightly increased to 0.35 at 6\u2005months. The Wilcoxon signed-rank test confirmed a statistically significant decrease in GPPASI over time (P &amp;lt; 0.05) from baseline, which persisted to 3\u2005months. Three relapses occurred during follow-up and all responded to acitretin reintroduction. Among the 12 patients tested, 7 carried the IL36RN c.80T&amp;gt;C(p.L27P) mutation in the homozygous state. Treatment response was slightly faster in these patients, but there were no statistically significant differences and the two groups converged by 3\u2005months. No adverse events were observed. Spesolimab demonstrated rapid and marked efficacy with excellent tolerability in GPP flares, regardless of IL36RN mutation status. Most cases had sustained remission, yet relapse occurred in a minority of patients. This calls for further research to optimize long-term and maintenance therapy.<\/jats:p>","DOI":"10.1093\/ced\/llag169","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T12:48:16Z","timestamp":1776430096000},"page":"1693-1698","source":"Crossref","is-referenced-by-count":0,"title":["Spesolimab for generalized pustular psoriasis: a\u00a0prospective study with a 6-month\u00a0follow-up"],"prefix":"10.1093","volume":"51","author":[{"given":"Emna","family":"Bahloul","sequence":"first","affiliation":[{"name":"Dermatology Department, Hedi Chaker University Hospital , Sfax ,","place":["Tunisia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-4032-8155","authenticated-orcid":false,"given":"Kmar","family":"Turki","sequence":"additional","affiliation":[{"name":"Dermatology Department, Hedi Chaker University Hospital , Sfax ,","place":["Tunisia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariem","family":"Nouri","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University , Sfax ,","place":["Tunisia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khadija","family":"Sellami","sequence":"additional","affiliation":[{"name":"Dermatology Department, Hedi Chaker University Hospital , Sfax ,","place":["Tunisia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Houda","family":"Hammami","sequence":"additional","affiliation":[{"name":"Dermatology Department, Habib Thameur Hospital , Tunis ,","place":["Tunisia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rim","family":"Chaabouni","sequence":"additional","affiliation":[{"name":"Dermatology Department, Hedi Chaker University Hospital , Sfax 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