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Capillary Malformations

(Nevus Flammeus; Port-Wine Stain)

by Denise M. Aaron, MD

Capillary malformations (also known as port-wine stains or nevi flammei) are present at birth and appear as flat, pink, red, or purplish lesions.

Port-wine stains are flat, reddish to purple lesions appearing anywhere on the body. Lesions become darker and more palpable with time (often becoming quite hyperplastic by late middle age), but the lateral extent increases only in proportion to the growth of the patient. Port-wine stains of the trigeminal area may be a component of the Sturge-Weber syndrome (in which a similar vascular lesion appears on the underlying meninges and cerebral cortex and is associated with seizures).

Diagnosis is made clinically. Imaging studies may be indicated, depending on findings, to diagnose associated syndromes (eg, Sturge-Weber syndrome).

Treatment with vascular lasers produces excellent results in many cases, especially if the lesion is treated as early in life as possible. The lesion can also be hidden with an opaque cosmetic cream prepared to match the patient’s skin color.

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