Purpose: To report corneal abnormalities associated with incontinentia pigmenti.
Case reports: Two girls, aged 2 1/2 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.