Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome (KLICK syndrome) is a rare cutaneous condition characterized by ichthyosis and keratoderma.

[1][2] It is an autosomal recessive disorder associated with a deletion in the transcription gene POMP, which codes proteasome maturation protein.

[3][4] This prevents the correct formation of filaggrin from profilaggrin.

[5] Sympotmatic treatment with keratolytics and retinoids is successful, but if treatment is stopped, symptoms recur.

[5] see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorder This Genodermatoses article is a stub.