Pathogenic variants in the ABCA12 gene cause various forms of autosomal recessive congenital ichthyosis (ARCI), such as severe harlequin ichthyosis (HI) and milder lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE) (8). The gene discussed is ABCA12; the disease is lamellar ichthyosis.