This autosomal recessive disorder of cornification occurs due to mutations in the serine protease inhibitor of Kazal type 5 (SPINK5) gene and results in ichthyosiform erythroderma which often manifests in the neonatal period, a peculiar abnormality of the hair shaft called trichorrhexis invaginata (bamboo hair) and allergic manifestations with elevation of IgE levels [181]. The gene discussed is SPINK5; the disease is congenital non-bullous ichthyosiform erythroderma.