171 Successful dupilumab treatment for ichthyotic and atopic features of Netherton syndrome

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Netherton syndrome (NS) is a rare, but severe autosomal-recessive ichthyosis syndrome characterized by a triad of ichthyosis, a hair shaft defect and atopic features. NS patients have a deficiency of serine protease inhibitor LEKTI in the stratum corneum, resulting in increased activity of serine proteases, accelerated desquamation of corneocytes and breakdown of the stratum corneum barrier, which accelerates percutaneous allergen sensitization. We performed a single-arm, prospective interventional study of the treatment with dupilumab, an interleukin (IL)-4 receptor α-antagonist that inhibits IL-4 and IL-13 signaling, for NS.