SPINK5 and Netherton syndrome: The unregulated actionsof both KLKs 5 and 7 result in Nethertonsyndrome (NS) development, a rare autosomal recessive disease thattriggers severe skin inflammation and scaling, since the loss of theinhibitor LEKTI activity is responsible for epidermal protease regulation.14 Another important point is that the mutationsin SPINK5 (serine protease inhibitor of Kazal-type 5) encoding LEKTIcause hair shaft defects and constant allergic manifestations.