Netherton syndrome: Temporary response to dupilumab.

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

Pediatric dermatology, vol.37, pp.1210-1211, 2020 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 37
  • 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
  • Marmara University Affiliated: Yes

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.