Among these genes are TGM1, CDSN, LOR, SPINK5, and FLG, each of whose mutation causes monogenic skin diseases characterized by abnormal epidermal barrier function, such as autosomal recessive congenital ichthyosis(44), peeling skin disease(45), keratoderma hereditaria mutilans(46), ichthyosis linearis circumflexa(47), and ichthyosis vulgaris(48) respectively. The gene discussed is SPINK5; the disease is autosomal recessive congenital ichthyosis.