Comel–Netherton (CN) syndrome is caused by a mutation in the serine protease inhibitor of Kazal type 5 (SPINK5) gene that encodes for lymphoepithelial kazal type–related inhibitor (LEKTI), whose deficiency is associated with elevated desmoglein 1 degradation and subsequent stratum corneum detachment (causing congenital ichthyosis or, in other patients, a scaly erythroderma), trichorrhexis invaginata, also known as “bamboo hair,” and HIE [11]. The gene discussed is SPINK5; the disease is ichthyosis.