1. Response to dupilumab in two children with Netherton syndrome: Improvement of pruritus and scaling.
- Author
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Süßmuth, K., Traupe, H., Loser, K., Ständer, S., Kessel, C., Wittkowski, H., and Oji, V.
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SYNDROMES in children , *ITCHING , *MACROPHAGE activation syndrome , *JUVENILE idiopathic arthritis - Abstract
Keywords: atopic dermatitis; genetics; genodermatoses; ichthyoses; immunology EN atopic dermatitis genetics genodermatoses ichthyoses immunology e152 e155 4 02/09/21 20210201 NES 210201 Dear editor Netherton syndrome (NTS) (MIM#256500) is an ichthyosis caused by I SPINK5 i mutations. Patients show erythema, scaling, severe pruritus, atopic manifestations, growth retardation and an increased susceptibility towards infections, making treatment of NTS challenging.1 Because of its clinical and pathophysiologic overlap with atopic dermatitis (AD) due to dysregulated protease activities2, dupilumab (interleukin (IL)-4/IL-13 antagonist) could be effective. Atopic dermatitis, genetics, genodermatoses, ichthyoses, immunology. [Extracted from the article]
- Published
- 2021
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