{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:23:43Z","timestamp":1791894223455,"version":"4.3.4"},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2024,10,12]],"date-time":"2024-10-12T00:00:00Z","timestamp":1728691200000},"content-version":"vor","delay-in-days":1258,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,6,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Patients with severe autosomal recessive congenital ichthyosis (ARCI) show a T helper 17\/interleukin 17 (Th17\/IL17) skewing in their skin and serum, resembling the inflammatory profile of psoriatic patients. Secukinumab, an IL-17A inhibitor, has shown clinical efficacy in patients with moderate-to-severe plaque psoriasis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>To test the clinical efficacy and safety of secukinumab in a paediatric patient with ATP-binding cassette subfamily A member 12 deficiency-related severe erythrodermic ARCI.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Materials &amp; Methods<\/jats:title>\n                  <jats:p>6-months therapeutic trial. During the first 4-weeks induction period, the patient received weekly subcutaneous injections of 150 mg secukinumab (five injections in total). During the following 20-weeks maintenance period, the patient was given a subcutaneous injection of 150 mg secukinumab every 4 weeks.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Result &amp; Discussion<\/jats:title>\n                  <jats:p>After the 6-months therapy period, there was a 48% reduction from the baseline Ichthyosis-Area-Severity-Index (-Erythema\/-Scaling) score. The treatment was well tolerated. Moreover, cytokine analysis revealed a reduction of keratinocyte-derived proinflammatory cytokines and an abrogation of Th17-skewing during therapy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Further studies are needed to evaluate the effects of the use of IL-17A inhibition in ARCI patients.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1002\/ski2.25","type":"journal-article","created":{"date-parts":[[2021,5,3]],"date-time":"2021-05-03T15:58:42Z","timestamp":1620057522000},"source":"Crossref","is-referenced-by-count":21,"title":["Efficacy and Safety of Secukinumab for the Treatment of Severe ABCA12 Deficiency-Related Ichthyosis in a Child"],"prefix":"10.1093","volume":"1","author":[{"given":"J.","family":"Yogarajah","sequence":"first","affiliation":[{"name":"Division of Paediatric Endocrinology, Diabetology and Metabolism, Department of Paediatrics, University Hospital Bern, 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