Incontinentia Pigmenti

Question 1

What is the inheritance?

Answer 1

X-linked dominant
Fatal in males in utero
Usually seen in females

Question 2

What is the first stage of IP?

Answer 2

Bullous
- erythematous, papular, bullous or vesicular lesions from birth to 4m
linear distribution and last for days or months

Question 3

What is the second stage of IP?

Answer 3

Verrucous for several months

Question 4

What is the third stage of IP?

Answer 4

Hyperpigmentation

- swirling, darkened areas following Blaschko’s line, occurring mainly on the trunk age 4m to 16yo

Question 5

What is the fourth stage of IP?

Answer 5

Atretic

- pale hairless atrophic linear streaks or patches

Question 6

What are minor crieria?

Answer 6

1. Hypodontia or anodontia or microdontia
2. Alopecia
3. Peripheral neovascularization of the retina
4. Mild ridging or pitting of nail or onychogryposis