Abstract

A female infant had erythematous vesicular skin lesions over the whole body and extremities in the early infancy. These skin lesions then changed to hyperpigmentation in whorls and splashes. Seizure attack was noted at one month old. Skin biopsy showed dyskeratosis, acanthosis, pigmenti incontinence, and massive infiltration of eosinophils. So Incontinentia pigmenti was confirmed. She also had hallmarks of retinal involvement, including peripheral retinal ischemia and neovascularization, which were similar to those seen in retinopathy of prematurity. Cryopexy was performed in her left eye and the lesions regressed.

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