AUTHOR=Barbati Federica , Giovannini Mattia , Oranges Teresa , Lodi Lorenzo , Barni Simona , Novembre Elio , Baldo Ermanno , Cristofolini Mario , Stagi Stefano , Ricci Silvia , Mori Francesca , Filippeschi Cesare , Azzari Chiara , Indolfi Giuseppe TITLE=Netherton Syndrome in Children: Management and Future Perspectives JOURNAL=Frontiers in Pediatrics VOLUME=9 YEAR=2021 URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.645259 DOI=10.3389/fped.2021.645259 ISSN=2296-2360 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.