Incontinentia Pigmenti

Incontinentia Pigmenti​

Incontinentia Pigmenti is 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 of Incontinentia Pigmenti

1- Skin manifestations

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.