Dermatology

Question 1

Port wine stain + Seizure suggests?

Answer 1

Sturge-Weber syndrome

Question 2

A port wine stain in which distribution may indicate Sturge-Weber Syndrome?

Answer 2

in the distribution of the ophthalmic or maxillary branches of the trigeminal nerve

Question 3

Features of Sturge Weber syndrome?

Answer 3

Neurological features: epilepsy, developmental delay, learning difficulties, stroke-like episodes

Ocular: glaucoma, buphthalmos (enlarged eye), vascular lesions of the conjunctiva/ episclera/ choroid/ retina

Port wine stain

Skull imaging: may demonstrate intracranial ‘tramline’ calcification

Leptomeningeal angiomata should be sought on MRI

Question 4

Port-wine stain + overgrowth of soft tissues and bones (hypertrophy) + venous/lymphatic malformations such as varicose veins

Answer 4

Klippel-Trenaunay syndrome