recessive X-linked ichthyosis

Last reviewed 01/2018

X linked ichthyosis is inherited as an X linked recessive with an incidence of 1 per 6000 males. It presents at birth or during infancy.

  • affected individuals are deficient in the enzyme steroid sulphatase which is necessary for the metabolism of cholesterol for the production of keratin in the skin
  • the condition is lifelong. It is most severe in cold weather and improves in the summer. It may worsen with age

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