Netherton syndrome: Temporary response to dupilumab

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

PEDIATRIC DERMATOLOGY, cilt.37, sa.6, ss.1210-1211, 2020 (SCI-Expanded) identifier identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 37 Sayı: 6
  • Basım Tarihi: 2020
  • Doi Numarası: 10.1111/pde.14362
  • Dergi Adı: PEDIATRIC DERMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.1210-1211
  • Anahtar Kelimeler: atopic eczema, genetic diseases, mechanisms, Ichthyosis
  • Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Hayır

Özet

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.