Humans with NIPAL4 deficient ARCI may be characterized phenotypically as non-bulbous congenital ichthyosiform erythroderma (CIE; fine white scale with erythema) or lamellar ichthyosis (LI; thick brown scale) as is typical for nonepidermolytic ARCI regardless of the genetic variant. The gene discussed is NIPAL4; the disease is autosomal recessive congenital ichthyosis.