X-linked ichthyosis

X-linked ichthyosis is an X-linked recessive genodermatosis associated with steroid sulfatase deficiency and elevated plasma Cholesterol sulfate. Generalized scaling is present at or shortly After birth, most prominently over the extremities, neck, trunk, and buttocks. It occurs only in males and may be associated with Testicular disease and corneal opacities.

Organ Affected

  • Skin System (Integumentary System)
  • Where it Occurs

  • Skin structure (body structure)
  • Entire skin (body structure)
  • Also Known As

  • Sex-linked ichthyosis
  • X-linked ichthyosis with steryl-sulfatase deficiency
  • X-linked recessive ichthyosis
  • Related ICD 10 COde

    ICD 10 Code Q80.1

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