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, 2020 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası:
  • Basım Tarihi: 2020
  • Doi Numarası: 10.1111/pde.14362
  • Dergi Adı: PEDIATRIC DERMATOLOGY

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.