Dupilumab in Paediatric Netherton Syndrome

Netherton Syndrome (NS) is a rare genodermatosis characterised by a triad of ichthyosiform erythroderma, an atopic diathesis, and trichorrhexis invaginata, for which no effective management is currently approved in Australia. Dupilumab has demonstrated considerable effectiveness in the treatment of atopic conditions in the paediatric population. We conducted a literature review to identify trends in disease aspects and their responsiveness to dupilumab treatment, with only four reports having follow-up beyond 12 months. To address this gap and provide insights into the long-term trajectory of dupilumab treatment in this population, we report two cases of NS treated with dupilumab over 20 and 25 months. This paper demonstrates that dupilumab may be a relatively safe and potentially effective option for targeting Th2-mediated inflammation though its overall sustained effectiveness may be variable.

Abstract from PubMed