ABSTRACT
Figure 20.2 ● Less frequently there is oral mucosa involvement with angiomas on tongue, gingivae and lips (Figure 20.7)
Extracutaneous findings ● Ipsilateral vascular malformation of the meninges and brain calcifications with epilepsy
and hemiplegia ● Mental retardation ● The eye may be involved ‘in toto’ (conjunctiva and iris, choroid and retina), resulting
in congenital glaucoma and major retinal abnormalities leading progressively to blindness
● Facial and body asymmetry
Course and complications
The prognosis is directly linked to the severity of CNS involvement. Strokes are rare.
