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).

Corneal asymmetry

The presence of a size difference between the left and right cornea.

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PMID (PMCID)
1890574	FEMALE	Child
	The ocular changes of incontinentia pigmenti achromians (hypomelanosis of Ito).
	Weaver RG Jr, Martin T, Zanolli MD. J Pediatr Ophthalmol Strabismus. 1991;28(3):160-3.
	We present a patient with incontinentia pigmenti achromians in whom the ocular abnormalities included the commonly reported exotropia, myopia, small optic nerve, and hypopigmentation of the fundus, as well as rarely reported corneal asymmetry, pannus, and atropic irides with irregular pupillary margins.