The lack of LEKTI expression in Netherton syndrome, a rare genetic skin disease characterized by congenital ichthyosis and severe allergic manifestations, indeed results in increased proteolytic activities of KLK5 and KLK7, which trigger an inflammatory process by activating protease-activated receptor-2 (PAR-2) and stimulating cytokine production [70] (Figure 3). Here, F2RL1 is linked to ichthyosis.