Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome (KLICK syndrome) is a rare cutaneous condition characterized by ichthyosis and keratoderma. It is an autosomal recessive disorder associated with a deletion in the transcription gene POMP, which codes proteasome maturation protein. This prevents the correct formation of filaggrin from profilaggrin. Sympotmatic treatment with keratolytics and retinoids is successful, but if treatment is stopped, symptoms recur.
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Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome (KLICK syndrome) is a rare cutaneous condition characterized by ichthyosis and keratoderma. It is an autosomal recessive disorder associated with a deletion in the transcription gene POMP, which codes proteasome maturation protein. This prevents the correct formation of filaggrin from profilaggrin. Sympotmatic treatment with keratolytics and retinoids is successful, but if treatment is stopped, symptoms recur.
has abstract
Keratosis linearis with ichthy ...... nt is stopped, symptoms recur.
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medical cause
OMIM id
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25,684,789
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994,691,470
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causes
Deletion in the POMP gene
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field
name
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
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OMIM
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Keratosis linearis with ichthy ...... nt is stopped, symptoms recur.
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label
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
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name
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
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