Affiliation:
1. Department of Dermatology Chang Gung Memorial Hospital Linkou Taoyuan Taiwan
2. Department of Dermatology Taipei Veterans General Hospital Taipei Taiwan
3. Faculty of Medicine School of Medicine National Yang Ming Chiao Tung University Taipei Taiwan
4. Department of Dermatology National Yang Ming Chiao Tung University Taipei Taiwan
5. Institute of Clinical Medicine National Yang‐Ming Chiao Tung University Taipei Taiwan
Abstract
SummaryDupilumab 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 “dupilumab,” “genodermatosis”, “Netherton syndrome”, “ichthyosis”, “epidermolysis bullosa” and “hyper‐IgE syndrome”. 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.
Cited by
9 articles.
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1. TH2-driven manifestations of inborn errors of immunity;Journal of Allergy and Clinical Immunology;2024-08
2. Dupilumab verbessert Juckreiz und Blasenbildung bei verschiedenen Subtypen der Epidermolysis bullosa;JDDG: Journal der Deutschen Dermatologischen Gesellschaft;2024-08
3. Dupilumab bessert kongenitale Ichthyose;JDDG: Journal der Deutschen Dermatologischen Gesellschaft;2024-06
4. Dupilumab improves itch and blistering in different subtypes of epidermolysis bullosa;JDDG: Journal der Deutschen Dermatologischen Gesellschaft;2024-04-28
5. Dupilumab improves congenital ichthyosis;JDDG: Journal der Deutschen Dermatologischen Gesellschaft;2024-04-11