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
2
Q
What are common associated abnormalities?
A
- Intellectual disability 70%
- Seizures 40%
- Microcephaly 25%
- Muscular hypotonia 15%
- Scoliosis
- Thoracic and limb deformitis
- strabismus, nystagmus 25%
- Cardiac defects 10%
3
Q
What is the inheritance of HI?
A
There is no evidence for genetic transmission
