Netherton Syndrome in Children: Management and Future Perspectives
Open Access
- 10 May 2021
- journal article
- review article
- Published by Frontiers Media SA in Frontiers in Pediatrics
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.This publication has 90 references indexed in Scilit:
- Topical treatment of ichthyosesDermatologic Therapy, 2013
- Mechanisms of Action of Topical Corticosteroids in PsoriasisInternational Journal of Endocrinology, 2012
- Ex-vivo Gene Therapy Restores LEKTI Activity and Corrects the Architecture of Netherton Syndrome-derived Skin GraftsMolecular Therapy, 2011
- IchthyosisAmerican Journal of Clinical Dermatology, 2009
- Comèl-Netherton syndrome defined as primary immunodeficiencyJournal of Allergy and Clinical Immunology, 2009
- Inducible Expression of the Proallergic Cytokine TSLP in Airway Epithelial Cells is Controlled by NFkappaBClinical Immunology, 2007
- Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cellsNature Medicine, 2006
- Netherton syndrome: report of identical twins presenting with severe atopic dermatitisEuropean Journal of Pediatrics, 2006
- Acute pancreatitis in a young girl with the Netherton syndromeJournal of Pediatric Surgery, 2005
- Effective treatment of Netherton's syndrome with 12% lactate lotionJournal of the American Academy of Dermatology, 1988