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FIGURE 69.14 Infant with acute hemorrhagic edema of infancy.

Hemangiomas
Hemangiomas of infancy (infantile hemangiomas) represent benign vascular
tumors that are present in approximately 3% of newborns and up to 10% of all
infants. These are seen more frequently in premature and low–birth-weight
infants and occur more commonly in girls than in boys. Superficial lesions
possess a red color, resembling a strawberry or raspberry. Deep lesions appear
soft, compressible, and often are faintly bluish. Mixed lesions may show features
of both superficial and deep hemangiomas. A precursor lesion may or may not be
noticeable immediately at birth. They typically undergo an early rapid growth
phase between 5 and 8 weeks old, continue to proliferative phase during the first
4 to 6 months, plateau in growth during the second 6 months, and then begin a
slow process of involution that lasts a few years. Beta-blockers, specifically
systemic propranolol and topical timolol, are used to treat severe hemangiomas of
infancy. Potential complications include bradycardia, hypotension, hypoglycemia,


bronchospasm, and hypothermia; however, overall safety, even in young infants,
has been good.
Although most lesions generally involute with little to no complications given
time, certain hemangiomas pose potential risks based on their anatomic location.
Rapidly enlarging hemangiomas near the eyes ( Fig. 69.15 ) may result in
amblyopia through obstruction of the visual axis (deprivation amblyopia) or
because of the compression of the eyeball itself (strabismus or anisometropia) and
require prompt intervention with systemic propranolol, steroids, or sometimes
surgery. Hemangiomas in a “beard” distribution—around the mouth, preauricular
areas, chin, or anterior neck—may indicate the presence of airway hemangiomas
and warrant further evaluation by direct laryngobronchoscopy or radiologic
imaging studies. Hemangiomas overlying the midline lower back may represent
markers for spinal dysraphism or tethered cord syndrome and warrant imaging.

Finally, large, segmental facial hemangiomas have been associated with
PHACE(S) syndrome, in which children suffer from P osterior fossa
malformations; facial H emangiomas; A rterial anomalies, including coarctation
of the aorta; structural C ardiac malformations; E ndocrinologic and structural E
ye abnormalities; and midline S ternal defects or supraumbilical raphe.
Intracranial vascular anomalies may predispose this subset of these children to an
increased risk for stroke. The corollary in the pelvic region is called LUMBAR
syndrome (lower body hemangioma and other cutaneous defects, urogenital
anomalies, ulceration, myelopathy, bony deformities, anorectal malformations,
arterial anomalies, and renal anomalies). These patients can also be at risk for
arterial as well as other urogenital and bony abnormalities ( Table 69.1 ).


FIGURE 69.15 Infant with rapidly enlarging hemangioma of the eye.

Hemangiomas occurring in any area, but especially the oral or genital areas,
may ulcerate and become secondarily infected, which may result in pain and
permanent scarring. Treatment with topical or oral antibiotics, oral propranolol,
topical timolol and wound care with nonadherent dressings, can be helpful in
managing these cases. Some may require treatment with a pulsed-dye laser.
Some vascular tumors, including kaposiform hemangioendotheliomas and
tufted angiomas, may resemble hemangiomas. These uncommon vascular tumors
may undergo sudden swelling with resulting hemolytic anemia,
thrombocytopenia, and congestive heart failure, resulting in a life-threatening
syndrome known as Kasabach–Merritt phenomenon. Patients with this syndrome


may require high doses of systemic corticosteroid or other chemotherapeutic
interventions to control these complications.
TABLE 69.1

COMPLICATIONS RELATED TO HEMANGIOMAS
Anatomic
location

Associated complication

Periocular
Beard area
Midline
prevertebral
Genital area
Large, facial
lesion

Amblyopia
Airway involvement
Tethered cord syndrome; spinal dysraphism

Large pelvic
area

Ulceration
PHACES (posterior fossa malformation; large facial
hemangioma; arterial anomalies; coarctation of the aorta or
other cardiac malformation; eye abnormalities; midline
sternal defects)
LUMBAR (lower body hemangioma, urogenital anomalies,
ulceration, myelopathy, bony deformities, anorectal
malformation, arterial anomalies, and renal anomalies)

DARKER AND LIGHTER SKIN LESIONS
Mastocytoma, Urticaria Pigmentosa
Children with mastocytomas or lesions of urticaria pigmentosa present with a
single yellow–tan–brown lesion that was present at or soon after birth
(mastocytoma) or multiple pigmented macules/papules that erupt during the first
year of life (urticaria pigmentosa). One important clue is a history of these lesions
becoming red ( Fig. 69.16 ), hive-like, or even blistered. The lesions may ooze
and form crusts, much like impetigo; however, they do not respond to topical
antibiotics.
On physical examination, appearance is variable. With extensive disease, the
surface may have a peau d’orange appearance. Some papules are yellow and are
easily mistaken for xanthomas. When lesions are tan to brown, they may be
mistaken for raised moles. The key finding is a positive Darier sign, which is
physical-induced erythema, swelling, and urtication secondary to scratching and