Autosomal recessive ichthyosis with hypotrichosis (ARIH, OMIM 610765), an inherited disorder linked to mutations in the ST14 gene coding for a serine protease matriptase, is characterized by the absence of the proteolytic activity of this type II transmembrane enzyme (Chen et al. 2014). This evidence concerns the gene ST14 and autosomal recessive congenital ichthyosis 11.