Pediatric emergency medicine trisk 2481 2481
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FIGURE 96.9 Nevus simplex (salmon patch). (Reprimted with permission from Goodheart HP.
Goodheart’s Photoguide of Common Skin Disorders . 2nd ed. Philadelphia, PA: Lippincott
Williams & Wilkins; 2003:1.)
Incontinentia Pigmenti
This is an X-linked dominant disorder with both skin and systemic lesions
affecting the eyes, CNS, and skeletal system. The disease presents at birth or
shortly after with an inflammatory vesicular or bullous rash that develops in crops
over the trunk and extremities. The cutaneous lesions have four phases
(inflammatory vesicles or bullae, verrucous lesions, whorled hyperpigmentation,
and hypopigmented patches) that may overlap and occur in irregular sequence.
Suspected cases should be referred to a dermatologist because of the potential for
systemic involvement.
Vascular Lesions
Salmon patch (nevus simplex ) is the most common vascular lesion of infancy.
It is a pale pink macular lesion that is found most commonly on the nape of the
neck, forehead, nasolabial region, or upper eyelids. Most resolve by the second
year of life. Lesions on the neck may persist for life ( Fig. 96.9 ).
Port-wine stains (nevus flammeus) present at birth as pink to purple macular
lesions than can vary tremendously in size ( Fig. 96.10 ). These lesions do not
fade with time. Klippel–Trénaunay–Weber syndrome should be considered when
the port-wine stain involves a lower limb. Consider Sturge–Weber syndrome
when infants present with unilateral facial port-wine stains in a trigeminal nerve
