Mutations in CERS3, PNPLA1, SDR9C7, and SULT2B1, have been identified in few families with ARCI presenting mainly LI phenotypes (Eckl et al., 2013; Grall et al., 2012; Heinz et al., 2017; Israeli et al., 2011; Shigehara et al., 2016). Here, CERS3 is linked to autosomal recessive congenital ichthyosis.