Dupilumab zur Behandlung von Genodermatosen: Eine systematische ubersicht

Dupilumab interferes with the signaling pathways of IL-4 and IL-13 and is effective in treating atopic dermatitis. Specific genodermatoses, including Netherton syndrome, epidermolysis bullosa pruriginosa, and hyper-IgE syndrome, are Th2 skewed diseases with activation of type 2 inflammation. We performed this systematic review to investigate the therapeutic role of dupilumab in the treatment of genodermatosis. A systematic search was conducted of the PubMed, Embase, Web of Science, and Cochrane databases from inception to December 13, 2021. The review included studies with relevant terms including & ldquo;dupilumab,& rdquo; & ldquo;genodermatosis, & rdquo;& ldquo;Netherton syndrome,& rdquo;& ldquo;ichthyosis,& rdquo;& ldquo;epidermolysis bullosa & rdquo;and & ldquo;hyper-IgE syndrome & rdquo;. The initial search yielded 2,888 results, of which 28 studies and 37 patients with genodermatosis were enrolled. The assessed genodermatoses included Netherton syndrome, epidermolysis bullosa pruriginosa, hyper-IgE syndrome, Hailey-Hailey disease, and severe eczema associated with genetic disorders. Most of the reported cases showed significant clinical improvement after the initiation of dupilumab treatment without major adverse events. Decreased immunoglobulin E levels and cytokine normalization have also been documented. In conclusion, Dupilumab may have a potential therapeutic role in certain genodermatoses skewed towards T helper 2 (Th2) immunity, including Netherton syndrome, epidermolysis bullosa pruriginosa, hyper-IgE syndrome, Hailey-Hailey disease, and severe eczema associated with some genetic disorders.

Automated summary

This systematic review investigated the therapeutic role of dupilumab in the treatment of genodermatosis. A systematic search identified 28 studies and 37 patients. The results showed that dupilumab was effective in treating certain Th2-skewed genodermatoses without major adverse events. These diseases included Netherton syndrome, epidermolysis bullosa pruriginosa, hyper-IgE syndrome, Hailey-Hailey disease, and severe eczema associated with genetic disorders. Decreased immunoglobulin E levels and cytokine normalization were also documented.