Port-wine stain

PWSs typically present at birth as well-demarcated red macules and

patches. PWS is not the correct term for the common congenital capillary stains located on the mid-face (forehead, glabella, tip of the nose
and philtrum), eyelids, and nape or occiput (Fig. 104.3A&B). Such
lesions are referred to as a nevus simplex or salmon patch; they are
also known as an angel kiss or aigrette on the forehead and a stork
bite on the neck. Many fade spontaneously between 1 and 3 years of
age, the facial ones more often than those in the nape area.
The growth of PWSs is commensurate with the childs growth. They
can be localized or have a segmental pattern, but they do not follow
the lines of Blaschko. Some are multifocal and widespread. Facial PWSs
are frequently distributed according to what has classically been considered the sensory trigeminal nerve dermatomes, with three areas
recognized: V1 (ophthalmic region: forehead and upper eyelid), V2
(maxillary region; Fig. 104.3C) and V3 (mandibular region).
Over time, PWSs (especially those affecting the V2 and V3 areas)
often develop a deeper red hue, changing from pinkish-red (at birth) to
purplish-red (in adulthood). Affected skin may thicken and become
nodular (Fig. 104.4), and superimposed pyogenic granulomas occasionally appear. In a study of 173 patients with PWSs, thickening was
observed in 11% (median age 32 years), nodularity in 24% (median age
44 years), and both in 6% (median age 45 years)7
. Overgrowth of the
maxilla (Fig. 104.5) and other facial bones underlying a PWS can also
occur, creating problems such as an open-bite deformity. Affected gums
and lips may enlarge, potentially resulting in epulides, gingival bleeding, macrocheilia and lip incompetence. Hypertrophic skin changes are
rare in PWSs on the trunk and limbs. Interestingly, when a child with
a PWS has atopic dermatitis, eczematous lesions may have a predilection for areas of skin affected by the CM.
PWSs are congenital in the vast majority of patients. However,

acquired PWSs have been described in adolescents and adults, and onset
of such lesions may be precipitated by trauma. Of note, early morphea
(especially the linear variant) occasionally presents with a red vascular
patch that mimics an acquired PWS7a.
A CM can be admixed with a network of blanched macules representing a nevus anemicus (Fig. 104.6), a phenomenon referred to as vascular twin spotting. The association of a CM with aberrant Mongolian
spots (dermal melanocytosis) (Fig. 104.7) or a nevus spilus (speckled
lentiginous nevus) is another example of twin spotting, which is referred
to as phakomatosis pigmentovascularis (PPV) (Table 104.4)
8,9.
A dorsal midline PWS (e.g. of the lumbosacral area) is occasionally
a hallmark for occult spinal dysraphism, particularly if the stain is
associated with other cutaneous markers of dysraphism (see Ch. 64).
In patients with clinically suspected dysraphism, ultrasound may be
useful in infants <24 months of age, but MRI is a more sensitive
study.