Long-term dupilumab therapy in Netherton syndrome with severe atopic manifestations: Case report and review of the literature

Özkaya E., Günay M. B., Babuna Kobaner G., Keskinkaya Z., Gökalp M. O.

Australasian Journal of Dermatology, vol.64, no.2, pp.272-277, 2023 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 64 Issue: 2
  • Publication Date: 2023
  • Doi Number: 10.1111/ajd.13986
  • Journal Name: Australasian Journal of Dermatology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE
  • Page Numbers: pp.272-277
  • Keywords: atopic dermatitis, hypohidrosis, hypotrichosis, ichthyosis, Netherton syndrome, pruritus, therapy-systemic
  • Çanakkale Onsekiz Mart University Affiliated: Yes

Abstract

We herein present a unique patient of Netherton syndrome (NS) with ichthyosis linearis circumflexa (ILC) lesions associated with severe atopic manifestations since infancy, showing different responses of atopic and ILC lesions to a 2-year dupilumab therapy. The atopic eczematous lesions and pruritus healed remarkably, dramatically improving the patient's quality of life, whilst the scalp hair showed a clinical and light microscopic improvement. The additional recovery in axillary/pubic/extremity hair growth, sweating and nail growth in the presented case was not previously reported in NS patients treated with dupilumab. However, dupilumab had no therapeutic effect on ILC lesions which were not pruritic and showed a treatment-independent wax and waned course.