Port-Wine Stain
Naevus Flammeus
Note the well defined unilateral border and trigeminal distribution of this port wine stain.
Features
Port-wine stains are almost invariably present from birth. However, they are often missed at first due to the normal hyperaemia of the neonatal skin. The lesions vary between light pink and dark red/purple in colour and range from a few millimetres in size to lesions involving most of the skin. The most common site to be involved is the face (in the region supplied by the trigeminal nerve), followed by the upper trunk. Although uncommon, Port-wine stains can occur anywhere on the body including the mucosae.
Nearly all lesions are unilateral and have a well defined edge. The lesions do not regress, and remain unchanged in size relative to the skin. They also tend to darken with age, and sometimes thicken and develop a cobblestone type appearance.
Port-wine stains are associated with underlying eye and brain abnormalities in 8-15%.
Incidence
The reported incidence is 0.1-2% in the newborn.
Aetiology
Congenital lesion of unknown aetiology. Due to its distribution in the trigeminal area, it was previously thought to be due to neurological birth trauma. This theory has now been discredited.
Differential Diagnosis
Treatment
Cosmetic camouflage can be used to cover disguise the lesion. Lasers, although not universally available on the NHS, can destroy the abnormal blood vessels with 2-3 treatments although it is worth noting that complete resolution is only achieved in 15% of patients. The laser treatments are painful, and usually require a GA in young children. In Aberdeen, laser therapy is offered to children when they become self conscious about their appearance.
