Port-Wine Stain (Nevus Flammeus)

Synonyms

Hemangioma; Nevus flammeus; Strawberry mark; Vascular nevus

Definition

A port-wine stain refers to a congenital angioma and is named as such due to the deep red hue rising from its vascularity. Found most commonly on the face, these grow proportionately with the body and darken over time from a pink to red-purple tone. Distribution is typically unilateral and respects the vertical midline. Facial lesions are typically distributed along the pattern of a branch of the trigeminal nerve, but frequent the forehead and upper eyelid, and hemihypertrophy of the face may develop. Oral lesions are present in 25 % of patients. Port-wine stains represent a classic feature of Sturge-Weber Syndrome, a phakomatosis with cutaneous, central nervous system and ocular abnormalities, in which ocular findings include glaucoma, abnormal plexus of episcleral vessels, iris or optic nerve coloboma, tortuous retinal vessels, and choroidal angioma.

Cross-References

• Bleu Nevus Disease