Furthermore, loss-of-function mutations in ST14 gene, encoding matriptase, in human patients with autosomal recessive ichthyosis and hypotrichosis (ARIH)/Ichthyosis, Follicular Atrophoderma, and Hypotrichosis (IFAH) and in horses with Naked Foal Syndrome indicate that the function of matriptase-prostasin proteolytic pathway in epithelial development may be evolutionarily conserved across mammalian species [14–18]. This evidence concerns the gene ST14 and autosomal recessive congenital ichthyosis 11.