recessive X-linked ichthyosis

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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

Click here for an example image of this condition


Last reviewed 01/2018