Port-wine stains (nevus flammeus, capillary malformation) aremalformations of the superficial capillaries of the skin. Theselesions are present at birth and should be considered permanent developmental defects. They do not enlarge after birth; any apparent increase in size is caused by growth of the child.A port-wine stain may be localized to any body surface, butfacial lesions are the most common. They are pink-red, sharplydemarcated macules and patches in infancy (Fig. 194-2). With time, they darken to a purple or port-wine color and maydevelop a pebbly or slightly thickened surface. Vascular blebsmay form within the lesions and become symptomatic or bleed.The most successful treatment modality in use is the pulsed dyelaser, which can result in 80% to 90% improvement in theselesions after a series of treatment sessions and can avoid future complications associated with vascular dilation. Treatment ismore effective if undertaken in infancy. Overgrowth of underlying bone can occur and is frequently seen with facial lesions.Affected patients often need maxillofacial intervention frommalalignment that develops.
Most port-wine stains occur as isolated defects and do not indicate systemic malformations. Rarely, they may suggest ocular defects or specific neurocutaneous syndromes. Sturge-Weber syndrome (encephalotrigeminal angiomatosis) may occur with a facial port-wine stain, usually in the cutaneous distribution of the first branch of the trigeminal nerve. Other features include leptomeningeal angiomatosis, mental retardation, seizures, hemiparesis contralateral to the facial lesions, ipsilateral intracortical calcification, and frequent ocular manifestations such as buphthalmos, glaucoma, angioma of the choroid, hemianoptic defects, and optic atrophy. Anticonvulsant therapy and neurosurgical procedures have been of value in some patients. Glaucoma can occur in association with port-wine stains located on the eyelid, even in the absence of Sturge-Weber syndrome, and these patients need lifelong monitoring of ocular pressures. Klippel-Trénaunay-Webersyndrome is characterized by the triad of capillary and venous malformations, venous varicosities, and hyperplasia of the soft tissues—and often bone—of the involved area. The lower limb is most commonly affected. A port-wine stain overlying the spine may rarely be a marker of spinal dysraphism or an intraspinal vascular malformation.
Figure 194-2 Port-wine stain (face).
A salmon patch (nevus simplex, stork bite, angel’s kiss) is avariant of nevus flammeus that is present in 70% of normal newborns. They are red, irregular, macular patches resultingfrom dilation of dermal capillaries and are usually found on thenape of the neck, the eyelids, and the glabella. Most of the faciallesions fade by 1 year of age, but lesions on the neck may persistfor life. Surveys of adult populations confirm the persistenceof the nuchal lesions in approximately 25% of the population.
ERYTHEMA MULTIFORME, STEVENS-JOHNSON SYNDROME, AND TOXIC EPIDERMAL NECROLYSIS