Journal of clinical immunology · 2024
Dupilumab in a 9-week-old with Netherton Syndrome Leads to Deep Symptom Control.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Vollmuth Yannik, Alelq Narjes Abdulhameed, Sattler Franziska, Schmidt Susanne, Hauck Fabian
Аннотация
PURPOSE: Netherton syndrome (NS) is a rare inborn error of immunity (IEI) with an incidence of approximately 1:200,000 and the phenotypic triad of trichorrhexis invaginate (bamboo hair), congenital ichthyosiform erythroderma, and multiple atopic manifestations. Treatment options especially in infants are scarce and generally not licensed.
METHODS: Case report of a 9-week-old infant with NS treated with dupilumab off-label.
RESULTS: We report rapid and sustained resolution of allergic inflammation, deep symptom control including normalization of the skin microbiome, and catch-up somatic and psychomotor development without adverse drug reactions.
CONCLUSION: Due to the high complication rate of NS, especially in the first years of life, we recommend treatment with dupilumab off-label immediately after the diagnosis has been established.
METHODS: Case report of a 9-week-old infant with NS treated with dupilumab off-label.
RESULTS: We report rapid and sustained resolution of allergic inflammation, deep symptom control including normalization of the skin microbiome, and catch-up somatic and psychomotor development without adverse drug reactions.
CONCLUSION: Due to the high complication rate of NS, especially in the first years of life, we recommend treatment with dupilumab off-label immediately after the diagnosis has been established.