ArticleFrontiers in immunology2022
Dupilumab improves clinical symptoms in children with Netherton syndrome by suppressing Th2-mediated inflammation.
Article in Frontiers in immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 19 citations in OpenAlex.
- Dupilumab in Inflammatory Skin Diseases: A Systematic Review.Biomolecules · 2023Pooled it
- Emerging Paediatric Uses of Dupilumab Beyond Approvals.Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology · 2026Review
- Club Cells in Nasal Epithelial Repair: Insights From an IL-13-Induced Inflammatory Model.Allergy · 2026Article
- Biologics and Small-Molecule Therapies in Netherton Syndrome: A Comprehensive Review.The Journal of dermatology · 2025Review
- Atopic dermatitis-induced failure to thrive and hypernatremic dehydration managed with dupilumab in a 5-month-old patient.JAAD case reports · 2025Article
- Expanding the potential of monoclonal antibodies against interleukin-4 and interleukin-13 in genodermatoses: A case series on the efficacy and safety of dupilumab in epidermolysis bullosa and ichthyosis.JAAD case reports · 2025Article
- Secukinumab as a Novel Treatment for Chronic Netherton Syndrome in a Young Adult.The American journal of case reports · 2025Article
- Inborn Errors of Immunity Presenting with Early-Onset Severe Atopy.Medicina (Kaunas, Lithuania) · 2025Review
- Correlation Between Systemic Immune Inflammatory Index and Suicidal Attempt in Patients with First-Episode Untreated Depression and its Risk Factors.Neuropsychiatric disease and treatment · 2025Article
- Clinical Improvement of Bullous Pemphigoid with Hyperkeratosis and Palmoplantar Keratoderma in Two Patients Treated with Dupilumab.Acta dermato-venereologica · 2024Article
- Interleukin-36 Is Highly Expressed in Skin Biopsies from Two Patients with Netherton Syndrome.Dermatopathology (Basel, Switzerland) · 2024Article
- TThe Journal of allergy and clinical immunology · 2024Review
- Article
- Dupilumab-resistant Netherton syndrome associated with a novel SPINK5 variant: c.575A>G (p.Asn192Ser).Anais brasileiros de dermatologiaArticle
- Dupilumab Reduces Pruritus in Twins With Sjögren-Larsson Syndrome.Pediatric dermatologyArticle
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: Netherton syndrome is a rare, life-threatening autosomal recessive genetic disorder with no effective treatment yet. Skin barrier dysfunction caused by Objective: To evaluate the clinical responses of patients with Netherton syndrome to dupilumab, an IL-4Rα antagonist, and identify changes in the Th1/2/17 pathway activity, skin barrier defect protein LEKTI expression after treatment. Methods: Four children with severe Netherton syndrome (aged 2 y to 4 y and 6 m) who were treated with dupilumab from January to June 2022 were evaluated at baseline, and at 4, 8, 12, 16, and 20 weeks after the start of dupilumab administration. Treatment response was assessed using the Eczema Area and Severity Index (EASI), the Numerical Rating Scale (NRS), the Dermatology Life Quality Index (CDLQI), and the Dermatitis Family Impact-questionnaire (DFI). Blood eosinophil counts, serum IgE levels and inflammatory cytokines were measured. The immunotyping of Th1/2/17 cells was performed by flow cytometry and cytokine expressions in T cell subsets were analyzed by single-cell RNA sequencing. In addition, expression of the LEKTI in skin lesions was evaluated by immunohistochemical analysis. Results: All four patients experienced clinical improvement, with significantly reduced EASI scores (by 75.0-83.9%) and NRS (by 87.5-90.0%) from baseline to 20 weeks of treatment. Improved quality of life scores were also seen for all patients, as measured by CDLQI and DFI. Serum IgE levels also fell by 75.6-86.9%. The serum Th2 cytokines IL-4, IL-5 and IL-13 were found at low level, with no significant changes during the treatment. However, Th2 cytokines expressed by T cells, especially IL-4, decreased at single-cell level after treatment ( Conclusion: Dupilumab may be an effective and safe treatment option in a subset of pediatric patients with Netherton syndrome, especially in improving itch and the quality of life. These effects were achieved in part by suppression of the Th2-mediated inflammation.
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