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Review
. 2021 May 10:9:645259.
doi: 10.3389/fped.2021.645259. eCollection 2021.

Netherton Syndrome in Children: Management and Future Perspectives

Federica Barbati  1 , Mattia Giovannini  2 , Teresa Oranges  3 , Lorenzo Lodi  1 , Simona Barni  2 , Elio Novembre  2 , Ermanno Baldo  4 , Mario Cristofolini  4 , Stefano Stagi  5 , Silvia Ricci  1 , Francesca Mori  2 , Cesare Filippeschi  3 , Chiara Azzari  1 , Giuseppe Indolfi  6
Affiliations
Review

Netherton Syndrome in Children: Management and Future Perspectives

Federica Barbati et al. Front Pediatr. .

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.

Keywords: Netherton syndrome; allergology; dermatology; immunology; management; pediatrics.

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Conflict of interest statement

CF received an honorarium from Sanofi-Regeneron for congress talks. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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