INTRODUCTION Also known as a port-wine stain, nevus flammeus is not a vascular neoplasm but a vascular capillary malformation composed of mature telangiectatic vessels. It can be seen commonly at birth as a discrete median and symmetrical vascular lesion that disappears within the first year of life. A more striking form of congenital nevus flammeus is asymmetric and persists throughout life. It can be isolated and unilateral, or associated with ocular and leptomeningeal vascular hamartomas as in the Sturge-Weber syndrome. A lighter-colored pink variant has been called nevus roseus and may be a distinct entity.
CLINICAL PRESENTATION Nevus flammeus presents as a flat purple or deep red vascular lesion that can vary from only a few millimeters in size to those covering vary large areas. On the face it is usually unilateral and in the distribution of one or more branches of the trigeminal nerve. Unlike other congenital eyelid vascular lesions it does not undergo spontaneous regression. In older adults nevus flammeus can undergo cavernous changes making them elevated and rather prominent. Pyogenic granuloma may arise within a nevus flammeus without any predisposing factors, as can rare cases of basal cell carcinoma. In the presence of Sturge-Weber syndrome associated ocular manifestations include diffuse choroidal hemangioma, ipsilateral glaucoma, and serous retinal detachment. Cases of acquired nevus flammeus have been described following trauma.
HISTOPATHOLOGY Both the salmon patch and port wine stains, encompassed by the term nevus flammeus, are characterized histologically by ectatic vessels of variable caliber within the dermis.
TREATMENT Treatment for light colored lesions may be limited to covering them with occlusive make-up.
However, Nd:YAG and pulsed dye laser therapy has been successful in treatment of these lesions. While complete eradication of the lesion is rarely accomplished, it can be made significantly lighter and less obvious. Intense pulsed light treatment has proven useful in cases resistant to laser therapy. The effectiveness of laser treatment is related to variations in skin thickness, being less beneficial in areas of thicker skin such as the midface area. Tissue hypertrophy that is sometimes associated with these lesions will remain. Surgical resection with full-thickness grafts are used less often than in the past.
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