Variants in DSG4 have previously been described in mice and rats with lanceolate hair phenotype, and in human patients with localized autosomal recessive hypotrichosis, as well as monilethrix-like hypotrichosis (Kljuic et al. 2003; Jahoda et al. 2004; Moss et al. 2004; Nagasaka et al. 2004; Rafiq et al. 2004; Messenger et al. 2005; John et al. 2006; Schaffer et al. 2006; Shimomura et al. 2006; Zlotogorski et al. 2006; Wajid et al. 2007). Here, DSG4 is linked to hypotrichosis.