Keratosis Linearis with Ichthyosis Congenita and Sclerosing Keratoderma, also known as keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome, is related to ichthyosis, congenital, autosomal recessive 1 and keratosis. An important gene associated with Keratosis Linearis with Ichthyosis Congenita and Sclerosing Keratoderma is POMP (Proteasome Maturation Protein). Affiliated tissues include skin, and related phenotypes are ichthyosis and palmoplantar keratoderma