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).

Retinal dysplasia

The presence of developmental dysplasia of the retina.

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PMID (PMCID)
6521980	FEMALE	Infant
	Incontinentia pigmenti associated with nasolacrimal duct obstruction.
	Smith B, Bedrossian EH Jr. Ophthalmic Surg. 1984;15(12):980-2.
	Ocular manifestations of incontinentia pigmenti have previously been described to include persistent hyperplastic primary vitreous, retinal dysplasia, retrolental fibroplasia, corneal opacities, cataract, optic atrophy, and strabismus.