Corneal abnormalities associated with incontinentia pigmenti

Purpose To report corneal abnormalities associated with incontinentia pigmenti. Case Reports Two girls, aged 21/2 and 5 years, with incontinentia pigmenti from one family underwent a complete ophthalmic examination. Results We found vortex (whorl-like) epithelial keratitis characterized by epithelial microcysts that stained with fluorescein and midstromal radial mild haziness in daughters of a family with incontinentia pigmenti. Conclusion Incontinentia pigmenti should be included in the differential diagnosis of children with keratitis, particularly if there is evidence of characteristic dermatologic or other systemic manifestations.