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).

Portal hypertension

Increased pressure in the portal vein.

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PMID (PMCID)
9161481	FEMALE
	[Association of hepatic adenomatosis and hepatoportal sclerosis in a woman with incontinentia pigmenti].
	Oberti F, Rifflet H, Flejou JF, Leclech C, Belghiti J, Rousselet MC, Cales P. Gastroenterol Clin Biol. 1997;21(2):147-51.
	We report the case of the association of three uncommon diseases in a young woman: incontinentia pigmenti, portal hypertension due to hepatoportal sclerosis, and liver adenomatosis.