Port-wine stain (syn. naevus flammeus)

Created: 25th April 2012 | Last Updated: 5th January 2015

Introduction

A port-wine stain is a vascular malformation of developmental origin with ectasia of superficial dermal capillaries. It is occasionally associated with congenital glaucoma, the Sturge-Weber syndrome (intracranial angiomas) and the Klippel-Trenaunay-Weber syndrome (limb hypertrophy).

This chapter is set out as follows:

History
Clinical findings
Images
Management

Related chapters

Haemangioma and other vascular tumours and malformations

History

A port-wine stain is almost always present at birth
The Sturge-Weber syndrome
- The usual cutaneous finding is a unilateral port-wine stain, involving roughly the areas served by the ophthalmic and maxillary divisions of the trigeminal nerve
- Epilepsy occurs in up to 90% of cases - once seizures start neurological deterioration with mental retardation can be rapid
- Patients without seizures fare much better
- Glaucoma and other eye complications arise in 50-60% of all cases
- Early referral is indicated
The Klippel-Trenaunay syndrome
- A rare disorder with a port-wine stain extending down the limb, increased limb size, varicose veins and other soft tissue swellings
- The condition is associated with many other developmental defects and high rates of deep vein thromboses and pulmonary emboli

Clinical findings

Distribution
- The face is the most commonly affected site
- The nape of the neck and upper trunk are other common sites, although any part of the body can be affected
Morphology
- Pink to deep red / purple patches
- Often unilateral with a distinct cut off
- Lesions tend to persist, darken and thicken with age

Images

Please click on images to enlarge, or choose to download. Images must only be used for teaching purposes and are not for commercial use. Notice and credit must be given to the PCDS and any other named contributor.

Download	Figure: 1 Port-wine stain and Sturge-Weber syndrome
Download	Figure: 2 Port-wine stain and Sturge-Weber syndrome The lesion had thickened over time
Download	Figure: 3 Port-wine stain
Download	Figure: 4 Port-wine stain
Download	Figure: 5 Klippel-Trenaunay syndrome and unaffected twin Port-wine stain (arrow), limb hypertrophy and varicose veins

Management

Facial lesions need early referral for the following reasons:
- To consider the possibility of the Sturge-Weber syndrome
- The cosmetic impact of such lesions can be substantial - referral to a dermatology department with laser facilities is appropriate
- Lesions on or near the eyelid can be associated with glaucoma - where the face is affected both above and below the eye the risk of glaucoma is particularly high. 40% of the cases of glaucoma arise in infancy, but glaucoma can also arise in adults and those at risk need early assessment and long-term follow up with an ophthalmologist
Sturge Weber UK - patient support group