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.