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.
Login or sign up to comment