Netherton Syndrome in Children: Management and Future Perspectives

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2021 May 27

PMID 34041207

Citations 10

Authors

Federica Barbati

Mattia Giovannini

Teresa Oranges

Lorenzo Lodi

Simona Barni

Elio Novembre

Ermanno Baldo

Mario Cristofolini

Stefano Stagi

Silvia Ricci

Francesca Mori

Cesare Filippeschi

Chiara Azzari

Giuseppe Indolfi

Affiliations

Soon will be listed here.

Abstract

Netherton syndrome (NS) is a genetic, multisystemic disease classically distinguished by a triad of clinical manifestations: congenital ichthyosiform erythroderma, hair shaft abnormalities, and immune dysregulation. Due to the complex pathogenesis of the disease, there are no specific therapies currently accessible for patients with NS. An early diagnosis is crucial to start the correct management of these patients. A multidisciplinary approach, including specialists in immunology, allergology, and dermatology, is necessary to set up the best therapeutic pathway. We conducted a review with the aim to summarize the different therapeutic strategies currently accessible and potentially available in the future for children with NS. However, given the limited data in the literature, the best-tailored management should be decided upon the basis of the specific clinical characteristics of the patients with this rare clinical condition. Further comprehension of the pathophysiology of the disease could lead to more efficacious specific therapeutic options, which could allow a change in the natural history of NS.

Citing Articles

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Kamis S, Yagci B Case Rep Oncol Med. 2025; 2025:5023552.

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Netherton Syndrome in Thai Children: A Report of Two Cases With a Literature Review.

Temboonnark P, Daengsuwan T Cureus. 2024; 16(6):e62718.

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Two Incidental Sibling Diagnoses of Netherton Syndrome in Separate Visits: A Case Report.

AlMoosawi S, Alkhanaizi S, Albaharna M, Khamdan F Cureus. 2024; 16(3):e56439.

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Interprofessional Collaboration: Differentiating Netherton Syndrome and Atopic Dermatitis in an African American Infant.

Park E, Besly K, Campos J, Rezk A Cureus. 2024; 16(3):e55905.

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[A case of neonatal Netherton syndrome].

Zhu Y, Jiang S, Zhang R, Cao Y, Zhang S Zhongguo Dang Dai Er Ke Za Zhi. 2024; 26(1):103-106.

PMID: 38269468 PMC: 10817731. DOI: 10.7499/j.issn.1008-8830.2307056.

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