{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T09:27:59Z","timestamp":1786440479191,"version":"build-2736575974"},"reference-count":33,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T00:00:00Z","timestamp":1786406400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antibodies"],"abstract":"<jats:p>Background\/Objectives: Congenital ichthyoses (CI) are inherited disorders of keratinization characterized by epidermal barrier dysfunction, abnormal desquamation, and variable degrees of inflammation. Increasing evidence suggests a role for IL-23\/Th17-mediated immune dysregulation in some CI subtypes, providing a rationale for targeted biologic therapies. Secukinumab, an anti-IL-17A monoclonal antibody, has emerged as a potential therapeutic option, although real-world evidence remains limited. This study evaluated the effectiveness of secukinumab in adult patients with severe CI. Methods: We performed a retrospective monocentric case series including eight adult patients with severe congenital ichthyosis treated with secukinumab for at least six months and followed for up to 56 months. Clinical outcomes included Investigator Global Assessment (IGA) scores for erythema, scaling, scalp involvement, and palmoplantar hyperkeratosis, together with pruritus assessed by Numeric Rating Scale (NRS). Results: Erythema and scaling showed most of their improvement during the first six months of treatment, after which mean clinical scores remained relatively stable throughout follow-up. At the last available assessment, improvement rates were 100% for erythema, 87.5% for trunk and limb scaling, 100% for scalp scaling, and 75% for palmoplantar hyperkeratosis. Hyperkeratosis showed a less pronounced response than inflammatory manifestations. Pruritus improved within the first 3\u20134 months of treatment and was sustained over time, with mean NRS scores decreasing from 6.5 at baseline to below 4 within 3\u20134 months. No discontinuations due to adverse events or lack of efficacy were recorded. Conclusions: Secukinumab was associated with clinically meaningful improvements in inflammatory manifestations of CI, particularly erythema and pruritus, while showing a more limited effect on hyperkeratotic features. These findings support the potential role of IL-17 inhibition in selected CI phenotypes.<\/jats:p>","DOI":"10.3390\/antib15040074","type":"journal-article","created":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T08:09:36Z","timestamp":1786435776000},"page":"74","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Real-World Effectiveness of Secukinumab in Congenital Ichthyoses: A Retrospective Monocentric Case Series"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-9270-1204","authenticated-orcid":false,"given":"Orsola","family":"Crespi","sequence":"first","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-7728-6832","authenticated-orcid":false,"given":"Umberto","family":"Santaniello","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Cangialosi","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ambra","family":"Bonvicino","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Ortoncelli","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1655-0395","authenticated-orcid":false,"given":"Francois","family":"Rosset","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Beauregard Hospital, Azienda USL della Valle d\u2019Aosta, Via L. Vaccari 5, 11100 Aosta, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7386-3219","authenticated-orcid":false,"given":"Luca","family":"Mastorino","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0098-1406","authenticated-orcid":false,"given":"Simone","family":"Ribero","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Quaglino","sequence":"additional","affiliation":[{"name":"Dermatologic Clinic, Department of Medical Science, University of Turin, 10126 Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/j.jaci.2016.07.019","article-title":"An IL-17\u2013dominant immune profile is shared across the major orphan forms of ichthyosis","volume":"139","author":"Paller","year":"2017","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1016\/j.jaci.2018.03.021","article-title":"Ichthyosis molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature","volume":"143","author":"Malik","year":"2019","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2157","DOI":"10.1016\/j.jid.2018.03.1523","article-title":"The Major Orphan Forms of Ichthyosis Are Characterized by Systemic T-Cell Activation and Th-17\/Tc-17\/Th-22\/Tc-22 Polarization in Blood","volume":"138","author":"Czarnowicki","year":"2018","journal-title":"J. Investig. Dermatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"319","DOI":"10.25259\/IJDVL_1749_2024","article-title":"Patients with congenital ichthyosis show differential activation of IL-23\/Th17 pathway among various disease subtypes\u2014A single-centre experience","volume":"92","author":"Kumar","year":"2026","journal-title":"Indian J. Dermatol. Venereol. Leprol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1016\/j.jaci.2022.02.010","article-title":"Understanding immune profiles in ichthyosis may lead to novel therapeutic targets","volume":"149","author":"Akiyama","year":"2022","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1186\/s13023-022-02430-6","article-title":"New developments in the molecular treatment of ichthyosis: Review of the literature","volume":"17","author":"Joosten","year":"2022","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Pe\u00f1a-Corona, S.I., Guti\u00e9rrez-Ruiz, S.C., Echeverria, M.D., Cort\u00e9s, H., Gonz\u00e1lez-Del, C.M., and Leyva-G\u00f3mez, G. (2023). Advances in the treatment of autosomal recessive congenital ichthyosis, a look towards the repositioning of drugs. Front. Pharmacol., 14.","DOI":"10.3389\/fphar.2023.1274248"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/j.jid.2018.08.027","article-title":"Profiling Immune Expression to Consider Repurposing Therapeutics for the Ichthyoses","volume":"139","author":"Paller","year":"2019","journal-title":"J. Investig. Dermatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1001\/jamadermatol.2020.1018","article-title":"Pathogenesis-Based Therapy With Repurposed Biologics for Monogenic Inflammatory Skin Disorders","volume":"156","author":"Paller","year":"2020","journal-title":"JAMA Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1111\/pde.15648","article-title":"Severe congenital ichthyosiform dermatosis in CHIME syndrome successfully treated with ixekizumab","volume":"41","author":"Flowers","year":"2024","journal-title":"Pediatr. Dermatol."},{"key":"ref_11","first-page":"829","article-title":"Dupilumab improves congenital ichthyosis","volume":"22","author":"Casetti","year":"2024","journal-title":"JDDG J. Dtsch. Dermatol. Ges."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1291","DOI":"10.1001\/jamadermatol.2025.3858","article-title":"Lamellar Ichthyosis Improvement With Acitretin and Dupilumab","volume":"161","author":"Nukaly","year":"2025","journal-title":"JAMA Dermatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1232","DOI":"10.1093\/ced\/llae552","article-title":"A striking hand phenotype and guselkumab efficacy in NIPAL4-linked autosomal recessive congenital ichthyosis","volume":"50","author":"Klapproth","year":"2025","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1007\/s00403-022-02325-3","article-title":"Secukinumab responses vary across the spectrum of congenital ichthyosis in adults","volume":"315","author":"Lefferdink","year":"2022","journal-title":"Arch. Dermatol. Res."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1093\/ced\/llae082","article-title":"Secukinumab therapy for paediatric patients with various phenotypes of congenital ichthyosis","volume":"49","author":"Albela","year":"2024","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e91","DOI":"10.1111\/jdv.20130","article-title":"Treatment of autosomal recessive congenital ichthyosis caused by a NIPAL4 variant with upadacitinib","volume":"39","author":"Gong","year":"2025","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"553794","DOI":"10.2147\/PTT.S553794","article-title":"Successful Treatment of Two Rare Pediatric Keratinization Disorders with Secukinumab: Epidermolytic Ichthyosis and PRP\u2013GPP Overlap","volume":"16","author":"Xu","year":"2026","journal-title":"Psoriasis Targets Ther."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1001\/jamadermatol.2020.1019","article-title":"Secukinumab Therapy for Netherton Syndrome","volume":"156","author":"Luchsinger","year":"2020","journal-title":"JAMA Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1111\/ajd.13880","article-title":"Secukinumab use in the treatment of Netherton\u2019s syndrome","volume":"63","author":"Gan","year":"2022","journal-title":"Australas. J. Dermatol."},{"key":"ref_20","first-page":"e25","article-title":"Efficacy and Safety of Secukinumab for the Treatment of Severe ABCA12 Deficiency-Related Ichthyosis in a Child","volume":"1","author":"Yogarajah","year":"2021","journal-title":"Ski. Health Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2288","DOI":"10.1111\/ced.15373","article-title":"Secukinumab significantly reduces inflammation but only mildly improves scaling in four cases of autosomal recessive congenital ichthyosis","volume":"47","author":"Subramani","year":"2022","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e946411","DOI":"10.12659\/AJCR.946411","article-title":"Secukinumab as a Novel Treatment for Chronic Netherton Syndrome in a Young Adult","volume":"26","author":"Buchukuri","year":"2025","journal-title":"Am. J. Case Rep."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1093\/ced\/llae040","article-title":"Transcriptomic response of peripheral blood mononuclear cells to secukinumab in epidermolytic ichthyosis","volume":"49","author":"Hou","year":"2024","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1111\/jpc.70044","article-title":"Treatment of Lamellar Ichthyosis in a 10-Year-Old Child With Secukinumab","volume":"61","author":"Mazzetto","year":"2025","journal-title":"J. Paediatr. Child Health"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1093\/bjd\/ljae420","article-title":"Biologics in congenital ichthyosis: Are they effective?","volume":"192","author":"Milesi","year":"2025","journal-title":"Br. J. Dermatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1111\/ajd.14197","article-title":"Biologics for inherited disorders of keratinisation: A systematic review","volume":"65","author":"Chen","year":"2024","journal-title":"Australas. J. Dermatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"110494","DOI":"10.1016\/j.clim.2025.110494","article-title":"Exploring TH17-mediated inflammation in epidermolytic ichthyosis: Clinical and mechanistic insight","volume":"277","author":"Tan","year":"2025","journal-title":"Clin. Immunol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.1111\/1346-8138.17923","article-title":"Biologics and Small-Molecule Therapies in Netherton Syndrome: A Comprehensive Review","volume":"52","author":"Morizane","year":"2025","journal-title":"J. Dermatol."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Mahajan, R., Bakshi, S., Kumar, A., De, D., and Handa, S. (2023). Case report: Interleukin-17 targeted biological therapy in netherton syndrome. Front. Pediatr., 11.","DOI":"10.3389\/fped.2023.1297658"},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Pontone, M., Giovannini, M., Filippeschi, C., Oranges, T., Pedaci, F.A., Mori, F., Barni, S., Barbati, F., Consonni, F., and Indolfi, G. (2022). Biological treatments for pediatric Netherton syndrome. Front. Pediatr., 10.","DOI":"10.3389\/fped.2022.1074243"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2176","DOI":"10.1056\/NEJMc1908531","article-title":"Imbalance in T-Helper 17 Cells and Targeted Therapy in an Infant with SAM-like Syndrome","volume":"381","author":"Vera","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1056\/NEJMoa1314258","article-title":"FIXTURE Study Group. Secukinumab in plaque psoriasis\u2014Results of two phase 3 trials","volume":"371","author":"Langley","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1007\/s12016-018-8702-3","article-title":"The Immunologic Role of IL-17 in Psoriasis and Psoriatic Arthritis Pathogenesis","volume":"55","author":"Blauvelt","year":"2018","journal-title":"Clin. Rev. Allergy Immunol."}],"container-title":["Antibodies"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4468\/15\/4\/74\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T08:32:45Z","timestamp":1786437165000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4468\/15\/4\/74"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,11]]},"references-count":33,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["antib15040074"],"URL":"https:\/\/doi.org\/10.3390\/antib15040074","relation":{},"ISSN":["2073-4468"],"issn-type":[{"value":"2073-4468","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,11]]}}}