Publication: Netherton syndrome: Temporary response to dupilumab
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Date
2020
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Publisher
WILEY
Abstract
Netherton syndrome (NS) is an orphan disease characterized by congenital ichthyosis, hair abnormalities, and atopy, with limited treatment options. We achieved temporary improvement only during the initial 6 weeks of treatment with dupilumab, which differs from the sustained improvement observed in 2 other recently published cases. Although the clinical presentation of atopy and increased pre-allergic cytokines in NS patients suggest that dupilumab may be beneficial, larger studies are required.
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Keywords
atopic eczema, genetic diseases, mechanisms, Ichthyosis