Multifocal hypopigmented retinal pigment epithelial lesions in incontinentia pigmenti.

Background: Incontinentia pigmenti (IP) can manifest with retinal detachment in children and can simulate retinoblastoma. The authors report a clinicopathologic correlation of IP and illustrate the retinal pigment epithelium (RPE) alterations that may be useful in establishing the diagnosis.

Methods: A 30-month-old reportedly healthy girl developed strabismus and was discovered to have total retinal detachment in the left eye.