Netherton Syndrome Subtypes Share IL-17/IL-36 Signature With Distinct IFN-α and Allergic Responses

September 2021 in “ Journal of Allergy and Clinical Immunology ”

Claire Barbieux, Mathilde Bonnet des Claustres, Matthias Fahrner, Evgeniya Petrova, Lam C. Tsoi, Olivier Gouin, F. Leturcq, P. Nicaise‐Roland, Christine Bole, Vivien Béziat, E. Bourrat, Oliver Schilling, Jóhann E. Guðjónsson, Alain Hovnanian

Netherton syndrome IL-17 IL-36 TH2 TH9 type I IFN epidermal proliferation epidermal differentiation IL-17A Janus kinase inhibitors precision medicine JAK inhibitors

TLDR Netherton syndrome has two subtypes with shared immune traits but different allergic and immune responses, suggesting targeted treatments.

The study on Netherton syndrome (NS) involved 13 patients and identified two subtypes, NS-ILC and NS-SE, both sharing an IL-17/IL-36 immune signature but exhibiting distinct immune responses. NS-ILC was characterized by a TH2-driven allergic response and complement activation, while NS-SE showed a TH9 axis and type I IFN pathway activation. The research highlighted abnormal epidermal proliferation and differentiation, with potential therapeutic targets such as IL-17A and Janus kinase inhibitors. The findings emphasized the need for precision medicine approaches to improve the quality of life for NS patients.