Hypomelanosis of Ito Flashcards

1
Q

What is the main cutaneous manifestation of HI?

A

Bizarre, patterned, hypopigmented macules arranged over body surface in sharply demarcated whorls, streaks and patches along the lines of Blaschko

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2
Q

What are common associated abnormalities?

A
  1. Intellectual disability 70%
  2. Seizures 40%
  3. Microcephaly 25%
  4. Muscular hypotonia 15%
  5. Scoliosis
  6. Thoracic and limb deformitis
  7. strabismus, nystagmus 25%
  8. Cardiac defects 10%
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3
Q

What is the inheritance of HI?

A

There is no evidence for genetic transmission

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