Incontinentia Pigmenti
Publish date 06-03-2016


Incontinentia Pigmenti is a hereditary systemic disease that is transmitted as X-linked disease

 

 

 

 

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.