Dupilumab in the treatment of genodermatosis: A systematic review

Summary 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....

Full description

Saved in:
Bibliographic Details
Published in:Journal der Deutschen Dermatologischen Gesellschaft 2023-01, Vol.21 (1), p.7-17
Main Authors: Wu, Po‐Chien, Dai, Ying‐Xiu, Li, Chia‐Lun, Chen, Chih‐Chiang, Chang, Yun‐Ting, Ma, Sheng‐Hsiang
Format: Article
Language:English
Subjects:
Atopic dermatitis
dupilumab
Eczema
Epidermolysis bullosa
Genetic disorders
Genodermatosis
Humans
hyper‐IgE syndrome
Ichthyosis
Immunoglobulin E
Lymphocytes T
Monoclonal antibodies
Netherton syndrome
Pemphigus, Benign Familial
Systematic review
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary 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 “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.
ISSN:1610-0379
1610-0387
DOI:10.1111/ddg.14924