2022
DOI: 10.1111/1346-8138.16645
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Ustekinumab therapy for Netherton syndrome

Abstract: Netherton syndrome (NS) is a rare disorder of cornification associated with high morbidity. It is caused by bi‐allelic mutations in SPINK5 encoding the serine protease inhibitor LEKTI. Previous studies have shown Th17 skewing with IL‐23 upregulation in NS, raising the possibility that targeting these inflammatory pathways may alleviate disease manifestations. We ascertained the therapeutic efficacy of six doses of ustekinumab administered to three patients with NS over a period of 13 months using the Ichthyosi… Show more

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