Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome

Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome, known as KLICK syndrome, is a rare inherited skin condition marked by thickened, hardened skin, or keratoderma, and dry, fish-scale-like skin, or ichthyosis. It is inherited in an autosomal recessive pattern and caused by a deletion in the POMP gene, which encodes a protein needed for proteasome maturation; the resulting defect disrupts the normal processing of profilaggrin into filaggrin, a protein essential to the skin barrier. Treatment relies on keratolytic agents and retinoids to manage the thickened and scaly skin, but these provide only temporary relief, and symptoms return once treatment stops, so ongoing management rather than a cure is the current standard of care.