Pediatric emergency medicine trisk 621

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


distribution. Because Sturge–Weber syndrome can involve seizures, intracranial
calcifications, and hemiparesis, infants with unilateral facial port-wine stains in
trigeminal nerve distribution need further assessment.

FIGURE 96.10 Nevus flammeus (port-wine stain). (Reprinted with permission from Nursing
management of the newborn. In: Ricci S, ed. Essentials of Maternity, Newborn, and Women’s

Health Nursing . 4th ed. Wolters Kluwer: Philadelphia, PA; 2017:585–643.)

Hemangiomas
Capillary hemangiomas (strawberry hemangioma) may be present at birth but
usually develop during the first few weeks of life. Lesions may present anywhere
on the body, starting as small, well-demarcated telangiectasis or macules that
develop into raised scarlet or purple tumors with distinct borders. Most capillary
hemangiomas grow rapidly the first 6 months of life, enter into a static period and
then recede, usually by 5 years of age ( Fig. 96.11 ). Cavernous hemangiomas
are deep-seated capillary hemangiomas that usually present at birth as diffuse
swelling with no color change or a bluish hue. Most spontaneously involute over
months to years.
Management . Hemangiomas that require intervention in the neonatal period are
those that may compromise vital structures, such as the eyes, nares, or auditory
canal, or lesions that by their location are susceptible to trauma, ulceration, and


secondary infection. Kasabach–Merritt syndrome, which presents as large,
rapidly enlarging hemangiomas associated with thrombocytopenia and
consumption coagulopathy, should be referred to appropriate specialists for
management. Monitor serial CBC and if evidence of significant
bleeding/coagulopathy, may require transfusions of red cells, platelets, or
coagulation factors.
Pigment Changes
Mongolian spots are poorly circumscribed lesions of bluish-gray maculae
generally located over the lumbosacral region, buttocks, and lower limb.
Mongolian spots are more commonly noted in infants of African-American,
Native American, Hispanic, and East Asian descent. Lesions usually fade during
the first year of life and do not require intervention ( Fig. 96.12 ).
Café-au-lait macules are round or oval, brown macular lesions varying in size

from less than 1 cm to greater than 20 cm. Though small, infrequent café-au-lait
macules are common in the general population, increases in number or size may
be a sign of neurocutaneous disease, such as neurofibromatosis. The clinician
should carefully examine these infants for other cutaneous or systemic signs of
disease ( Fig. 96.13 ).


FIGURE 96.11 Capillary hemangioma (strawberry hemangioma). (Courtesy of Dean John
Bonsall, MD. In: Chung EK, Boom JA, Datto GA, et al., eds. Visual Diagnosis in Pediatrics .
Philadelphia, PA: Lippincott Williams & Wilkins; 2006. With permission.)