{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T17:13:45Z","timestamp":1789665225049,"version":"4.0.1"},"reference-count":10,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T00:00:00Z","timestamp":1739232000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T00:00:00Z","timestamp":1739232000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ital J Pediatr"],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>Generalized Pustular Psoriasis (GPP) is an autoinflammatory, multisystemic disease, characterized by widespread eruption of neutrophilic pustules on erythematous base, accompanied by systemic symptoms such as fever, leukocytosis, arthralgia, and general malaise. Globally, the disease is rare, particularly in children. If not adequately diagnosed and treated, systemic inflammation and multiorgan involvement can be life-threatening. The pathogenesis of GPP mainly involves the innate immune system, with inflammatory processes and neutrophil activation driven primarily by IL-36, but also by IL-1, TNF-alpha, IL-17\u00a0A. In particular, IL-17\u00a0A acts as a potent inducer of neutrophil recruitment. We report the case of a 7-years-old girl with GPP successfully treated with Ixekizumab, an IL-17\u00a0A antagonist.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Case presentation<\/jats:title>\n            <jats:p>A 7-years-old girl with an history of plaque psoriasis came to our attention for the sudden appearance of erythematous patches surmounted by pustules on the trunk and lower limbs, following repeated episodes of purulent tonsillitis. We started therapy with cyclosporine at a dosage of 3,5\u00a0mg\/kg\/day, with no clinical benefit and progression of manifestations to a sub-erythrodermic state after 2 weeks. Blood tests showed neutrophilic leukocytosis, and the patient experienced hyperpyrexia and malaise. Since ixekizumab was recently approved for pediatric use in patients with moderate to severe plaque psoriasis, we started therapy with 80\u00a0mg Ixekizumab, combined with prednisone at a dosage of 12.5\u00a0mg\/day, gradually tapered until discontinuation after 15 days. A second dose of Ixekizumab 40\u00a0mg was administered at week-4, according to the indication of ixekizumab in pediatric plaque psoriasis. At week-8 the patient achieved complete remission of skin manifestations and normalization of blood count. After achieving a stable remission, at week 36 we decided to increase the administration interval to 6 weeks. The patient is still on therapy with ixekizumab 40\u00a0mg every 6 weeks, maintaining complete remission during a 52-week follow-up, without safety concerns.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>This report supports the use of ixekizumab as a safe and effective option, both in the short and long-term, in the treatment of GPP, even at pediatric age. Larger studies are needed to confirm this positive, real-life experience.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/s13052-024-01835-2","type":"journal-article","created":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T18:15:25Z","timestamp":1739297725000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Ixekizumab as a successful treatment in pediatric generalized pustular psoriasis"],"prefix":"10.1186","volume":"51","author":[{"given":"Maria","family":"Esposito","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Antonetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Vagnozzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"De Berardinis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Bertelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Brancati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Concetta","family":"Fargnoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,2,11]]},"reference":[{"issue":"11","key":"1835_CR1","doi-asserted-by":"publisher","first-page":"1792","DOI":"10.1111\/jdv.14386","volume":"31","author":"AA Navarini","year":"2017","unstructured":"Navarini AA, Burden AD, Capon F, Mrowietz U, Puig L, K\u00f6ks S, Kingo K, Smith C, Barker JN. 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Psychometric validation of the generalized pustular psoriasis physician global assessment (GPPGA) and generalized pustular psoriasis area and severity index (GPPASI). J Eur Acad Dermatol Venereol. 2023;37(7):1327\u201335.","journal-title":"J Eur Acad Dermatol Venereol"},{"issue":"10","key":"1835_CR7","doi-asserted-by":"publisher","first-page":"981","DOI":"10.1056\/NEJMc1811317","volume":"380","author":"H Bachelez","year":"2019","unstructured":"Bachelez H, Choon SE, Marrakchi S, Burden AD, Tsai TF, Morita A, Turki H, Hall DB, Shear M, Baum P, Padula SJ, Thoma C. Inhibition of the Interleukin-36 pathway for the treatment of generalized Pustular Psoriasis. N Engl J Med. 2019;380(10):981\u20133.","journal-title":"N Engl J Med"},{"issue":"2","key":"1835_CR8","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1016\/j.jaad.2021.10.028","volume":"87","author":"M Sachdeva","year":"2022","unstructured":"Sachdeva M, Rankin BD, Mufti A, Yeung J, Vender R, Luca NJ, Miettunen P, Stevenson R, Devani AR, Prajapati VH. Management of pediatric generalized pustular psoriasis using biologics: an evidence-based review. J Am Acad Dermatol. 2022;87(2):484\u20136.","journal-title":"J Am Acad Dermatol"},{"issue":"3","key":"1835_CR9","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1159\/000538072","volume":"240","author":"F Prignano","year":"2024","unstructured":"Prignano F, Atzori L, Bellinato F, Damiani G, Galeone C, Mariani P, Potenza C, Scopinaro A, Piaserico S, Fabbrocini G. Italian Delphi GPP Group. 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J Dermatol. 2017;44(4):355\u201362.","journal-title":"J Dermatol"}],"container-title":["Italian Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13052-024-01835-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13052-024-01835-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13052-024-01835-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,11]],"date-time":"2025-02-11T18:15:26Z","timestamp":1739297726000},"score":1,"resource":{"primary":{"URL":"https:\/\/ijponline.biomedcentral.com\/articles\/10.1186\/s13052-024-01835-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,11]]},"references-count":10,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["1835"],"URL":"https:\/\/doi.org\/10.1186\/s13052-024-01835-2","relation":{},"ISSN":["1824-7288"],"issn-type":[{"value":"1824-7288","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,2,11]]},"assertion":[{"value":"24 May 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 December 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 February 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written consent has been obtained from the patient\u2019s parents for the use of the photos and the clinical history.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"M. Esposito has served as a speaker\/board member for Abbvie, Almirall, Biogen, Celgene, Eli Lilly, Janssen, Leo Pharma, Novartis, Sanofi, UCB. P. Antonetti has no conflicts of interests to declare. E. Vagnozzi has no conflicts of interest to declare. A. De Berardinis has no conflicts of interest to declare. R. Bertelli has no conflicts of interest to declare. F. Brancati has no conflicts of interest to declare. M. C. Fargnoli has served as consultant\/advisor, received speaker honoraria and\/or grants, and\/or is investigator for AMGEN, Almirall, Abbvie, Boehringer-Ingelheim, BMS, Galderma, Kyowa Kyrin, Incyte, LEO Pharma, Pierre Fabre, UCB, Lilly, Pfizer, Janssen, MSD, Novartis, Sanofi, Regeneron, Sun Pharma, Takeda.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"41"}}