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).

Blindness

Blindness is the condition of lacking visual perception defined as visual perception below 3/60 and/or a visual field of no greater than 10 degress in radius around central fixation.

Total: 2

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PMID (PMCID)
19213518	FEMALE	Child
	Incontinentia pigmenti: a rare cause of retinal vasculitis in children.
	El Fekih L, Hmaied W, Souissi K, Nasri H, Derbel F, Hamdi A. Tunis Med. 2008;86(12):1079-81.
	Early diagnosis and management of retinal changes in incontinentia pigmenti is essential because they may be serious leading to blindness.
3390422	FEMALE	Infant, Newborn
	Incontinentia pigmenti: the development of pseudoglioma.
	Brown CA. Br J Ophthalmol. 1988;72(6):452-5.
	A case is described of incontinentia pigmenti in an infant with relatively normal retinae at seven days after birth who went on to total blindness by three months.