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).

Hypertension

The presence of chronic increased pressure in the systemic arterial system.

Total: 4

(per page)

PMID (PMCID)
15884011	FEMALE	Infant, Newborn
	Unilateral acheiria and fatal primary pulmonary hypertension in a girl with incontinentia pigmenti.
	Hayes IM, Varigos G, Upjohn EJ, Orchard DC, Penny DJ, Savarirayan R. Am J Med Genet A. 2005;135(3):302-3.
	We describe a newborn girl with incontinentia pigmenti (IP, MIM308300), unilateral acheiria, and fatal primary pulmonary hypertension.
15789024	FEMALE	Infant, Newborn
	Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.
	Godambe S, McNamara P, Rajguru M, Hellmann J. J Perinatol. 2005;25(4):289-92.
	Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.
15789024	FEMALE	Infant, Newborn
	Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.
	Godambe S, McNamara P, Rajguru M, Hellmann J. J Perinatol. 2005;25(4):289-92.
	We report a neonate with a diagnosis of incontinentia pigmenti who presented at birth with pulmonary hypertension.
11207974	FEMALE	Infant
	Incontinentia pigmenti: a case associated with cardiovascular anomalies.
	Miteva L, Nikolova A. Pediatr Dermatol. 2001;18(1):54-6.
	We report an isolated case of incontinentia pigmenti in a 6-month-old girl in association with tricuspid insufficiency, an abnormal shunt of the right pulmonary vein into the superior vena cava, and pulmonary hypertension.