Moreover, Nrf2-mediated reduction of oxidative stress might contribute to the beneficial effect of Nrf2 activation, as seen previously in a mouse model for palmoplantar keratoderma, a characteristic feature of pachyonychia congenita, which results from mutations in the K6, K16 or K17 genes (Kerns et al., 2016). Here, NFE2L2 is linked to pachyonychia congenita.