PROLIFERATIVE RETINOPATHY ASSOCIATED WITH INCONTINENTIA PIGMENTI

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Abstract
An 8-year-old white female presented with retinal vascular changes of fibrovascular proliferation and avascularity of the peripheral retina that suggested a diagnosis of incontinentia pigmenti. These findings prompted a systemic evaluation, and the features of this disease were confirmed, including a history of recurring vesicles on the lower extremities of her sister in infancy. Associated systemic findings in the patient included alopecia, apigmented skin lesions, conical teeth, and a confirmatory biopsy. This case illustrates that a clinical recognition of eye findings can be of assistance in correctly diagnosing such forme fruste cases.

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