Netherton 综合征：发病机制与临床意义的新认识。
Netherton Syndrome: Insights into Pathogenesis and Clinical Implications.
机构信息
Service of Dermatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Service of Dermatology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
出版信息
J Invest Dermatol. 2020 Jun;140(6):1129-1130. doi: 10.1016/j.jid.2019.11.007.
Netherton syndrome (NS) is a rare skin disorder involving the skin, hair, and immune system. Pathological manifestations are due to unopposed kallikrein peptidase activity because of a SPINK5 gene deficiency. In their article, Gouin et al. explore the role of kallikrein 14 in the stratum granulosum, defining it as an important player implicated in the pathogenesis of NS hair shaft anomalies.
Netherton 综合征（NS）是一种罕见的皮肤疾病，涉及皮肤、毛发和免疫系统。其病理表现是由于 SPINK5 基因缺陷导致无拮抗的激肽释放酶活性。Gouin 等人在他们的文章中探讨了激肽释放酶 14 在颗粒层中的作用，将其定义为参与 NS 毛发异常发病机制的重要因素。
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