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Secukinumab Therapy for Netherton Syndrome
Isabelle Luchsinger1,2, Nicole Knöpfel1,2, Martin Theiler1,2
1Pediatric Skin Center, Department of Dermatology, University Children's Hospital Zurich, Zurich, Switzerland.
JAMA Dermatology
|May 28, 2020
Summary
Secukinumab, an IL-17 antagonist, significantly improved Netherton syndrome (NS) symptoms in a small case series. This targeted therapy showed marked skin improvement, particularly in pediatric patients, offering a promising new treatment option for this severe genetic disorder.
Area of Science:
- Dermatology
- Immunology
- Genetics
Background:
- Netherton syndrome (NS) is a severe genetic disorder of cornification with limited treatment options.
- Recent research indicates an overactive helper T cell (TH) 17/interleukin 23 (IL-23) pathway in NS, suggesting IL-17 as a therapeutic target.
Purpose of the Study:
- To assess the clinical effectiveness of secukinumab, an IL-17 antagonist, in treating patients with Netherton syndrome.
Main Methods:
- A case series involving 4 patients (2 children) with severe NS treated with secukinumab.
- Evaluation included skin IL-17 expression, serum cytokines (CCL20), Ichthyosis Area and Severity Index (IASI), Dermatology Life Quality Index (DLQI), and 5-D itch scale.
Main Results:
- All patients showed increased IL-17 in lesional skin and elevated serum CCL20.
- After 3 months, IASI scores decreased by 44-88%, DLQI by 40-76%, and itch scores by 27-62%.
- Marked improvement was observed in erythrodermic phenotypes; refractory palmoplantar eczema and candidal nail infections occurred in some patients. No severe adverse events were reported.
Conclusions:
- Anti-IL-17 therapy with secukinumab demonstrated significant cutaneous improvement in Netherton syndrome patients.
- The study highlights the potential of targeting the IL-17 pathway for NS treatment, especially in pediatric cases with erythrodermic phenotypes.
- Further research is warranted to confirm long-term efficacy and safety.
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