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Phakomatosis pigmentovascularis: a case report

Poster Details

First Author: S.Ganatra INDIA

Co Author(s):    M. Patel                    

Abstract Details


To report a case of Phakomatosis pigmentovascularis (PPV) which is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi.


M and J Western Regional Institute of Ophthalmology, Ahmedabad, Gujarat, India


Case report


A 7-year-old female child presented to us with facial port-wine stains distributed along the 3 branches of trigeminal nerve, which suggested the existence of Sturge Weber Syndrome(SWS). The diagnosis of Ota nevus was made by the bilateral scleral melanocystosis. Increased intraocular pressure, megalocornea, and pathologic optic disc cupping supported the diagnosis of bilateral glaucoma.


The simultaneous coexistence of PPV with SWS, Ota nevus, and congenital glaucoma is rare. Detailed clinical examination to exclude other ocular abnormalities or extraocular involvement are necessary.

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