Incontinentia Pigmenti

A hereditary systemic disease that is transmitted as X-linked disease. It is lethal for male fetus during pregnancy thus it affects born female during the first few years.

Features

Erythema and bullae at extremities with hyperpigmented skin lesions in a Christmas tree pattern on the trunk.

2- Neurological features:

Epilepsy, mental retardation and Hydrocephalus.

3- Ocular manifestations:

Cataract, Optic atrophy, foveal hyoplasia and poorly developed peripheral retina which causes retinal hemorrhage, vitreous hemorrhage, abnormal blood vessels and tractional retinal detachment.

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