Incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS).

Hypopigmented streaks

Total: 1

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PMID (PMCID)
7962735	FEMALE	Infant, Newborn
	Incontinentia pigmenti: three cases with unusual features.
	Sahn EE, Davidson LS. J Am Acad Dermatol. 1994;31(5 Pt 2):852-7.
	One child with skin, ophthalmologic, and dental findings had atrophic, hypopigmented streaks on her legs by the age of 2 1/2 years that were consistent with the fourth stage of incontinentia pigmenti.