X-linked recessive ichthyosis

X-linked recessive ichthyosis

This recessive disorder, X-linked ichthyosis, is found in males.
Clinically seen as a more severe variant of ichthyosis vulgaris, with relative but not complete sparing of the flexures, it commonly shows thickened brown scales. The palms and soles may show some hyperkeratosis. Corneal opacities are found in some patients.

Treatment:

  • Topical hydroxy acids such as tartaric acid 5 % in an ointment base are sometimes helpful, but are generally not as effective in this condition as in lamellar ichthyosis.
  • Agents that may be useful include ammonium lactate 12 % lotion and topical 20 % urea.
  • Topical 10 % cholesterol in a cream base has also been reported to be effective.
  • Systemic retinoids such as etretinate may be quite effective, but as treatment must be continued life long, treatment should be started with systemic retinoids only in the most severe cases.
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