Ontology highlight
ABSTRACT: Importance
Netherton syndrome (NS) is a rare, severe genetic disorder of cornification with high morbidity. Treatment for NS has been notoriously difficult. Recent studies showed an upregulated helper T cell (TH) 17/interleukin 23 (IL-23) pathway in NS, suggesting the possibility of treatment strategies that target IL-17.Objective
To evaluate the clinical response of NS to treatment with the IL-17 antagonist secukinumab.Design, setting, and participants
This case series study reports the experience of compassionate use therapy with secukinumab in 4 patients with severe NS, including 2 children, from December 1, 2018, to December 1, 2019, with 3 patients still undergoing treatment at the time of final analysis. Data were analyzed from December 1, 2018, to December 1,
SUBMITTER: Luchsinger I
PROVIDER: S-EPMC7254452 | biostudies-literature | 2020 Aug
REPOSITORIES: biostudies-literature