PURPOSE: To report corneal abnormalities associated with incontinentia pigmenti. CASE REPORTS: Two girls, aged 2 1/4 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.
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