Abstract
To the Editor: Netherton syndrome (NS) is a rare autosomal recessive ichthyosis due to loss-of-function mutations in SPINK5 encoding LEKTI.1 Patients with NS typically present with ichthyosis linearis circumflexa (NS-ILC) or scaly erythroderma (NS-SE) associated with trichorrhexis invaginata and severe atopic manifestations with high serum immunoglobulin E levels.2 Current NS treatment is only symptomatic. Recent studies showed that patients with NS display a prevailing T helper (Th) type 17 immune response,3,4 suggesting that blocking the Th17 axis could be a therapeutic strategy.
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