Incontinentia Pigmenti
Neurocutaneous syndromes
inherited ectodermal disorder
features dermatologic, dental, and ocular abnormalities.
functional mosaicism caused by random X-inactivation of an X-linked dominant gene that is lethal in males (IKK-gamma/NEMO gene).
Phase 1: erythematous linear streaks and plaques of vesicles
Phase 2: hyperkeratotic
Phase 3: Hyperpigmentation in blaschko lines
Phase 4: Hypopigmentation (hairless, sweatless)
inherited ectodermal disorder
features dermatologic, dental, and ocular abnormalities.
functional mosaicism caused by random X-inactivation of an X-linked dominant gene that is lethal in males (IKK-gamma/NEMO gene).
Phase 1: erythematous linear streaks and plaques of vesicles
Phase 2: hyperkeratotic
Phase 3: Hyperpigmentation in blaschko lines
Phase 4: Hypopigmentation (hairless, sweatless)