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