Netherton syndrome: Temporary response to dupilumab


Aktas M., Salman A., Apti Sengun Ö., Comert Ozer E., Hosgoren Tekin S., Akin Cakici O., ...More

PEDIATRIC DERMATOLOGY, vol.37, no.6, pp.1210-1211, 2020 (SCI-Expanded, Scopus)

  • Publication Type: Article / Article
  • Volume: 37 Issue: 6
  • Publication Date: 2020
  • Doi Number: 10.1111/pde.14362
  • Journal Name: PEDIATRIC DERMATOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE, MEDLINE
  • Page Numbers: pp.1210-1211
  • Keywords: atopic eczema, genetic diseases, mechanisms, Ichthyosis
  • Acibadem Mehmet Ali Aydinlar University Affiliated: No

Abstract

Netherton syndrome (NS) is an orphan disease characterized by congenital ichthyosis, hair abnormalities, and atopy, with limited treatment options. We achieved temporary improvement only during the initial 6 weeks of treatment with dupilumab, which differs from the sustained improvement observed in 2 other recently published cases. Although the clinical presentation of atopy and increased pre-allergic cytokines in NS patients suggest that dupilumab may be beneficial, larger studies are required.