Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
Medical condition / From Wikipedia, the free encyclopedia
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome (KLICK syndrome) is a rare cutaneous condition characterized by ichthyosis and keratoderma.[1][2]
Quick Facts Specialty, Causes ...
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome | |
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Specialty | Dermatology |
Causes | Deletion in the POMP gene |
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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]