Common Ichthyosis

X-Linked Ichthyosis (Non-syndromic)

  • Beginning: Childhood
  • Clinical picture: Mild
  • Non-syndromic: Does not affect other organs besides the skin.

X-linked recessive ichthyosis only affects males and occurs in 1 in 2,000 – 6,000 births. Desquamation begins in the neonatal period (first month of life) and is generally more prominent on the extensor surfaces, although there is also significant involvement of the flexor areas.

Although the extent and degree of desquamation are variable, X-linked ichthyosis can generally be distinguished from vulgar ichthyosis by clinical criteria. The latter usually presents hyperlinear palms and soles, follicular keratosis (sandpaper-like skin in certain locations) and a family history of atopy.

X-linked ichthyosis usually produces more severe involvement, with larger, adherent, brownish-colored scales. In adult patients, there may be opacities in the horny layer of the eye that do not affect vision and may be present in female carriers as well.

This disease is caused by the congenital deficiency of the steroid enzyme sulfatase, which causes these sulfates to present high values ​​in the serum, the epidermis and the scales.

Women act as carriers of the disease but have no symptoms.