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 and weeks old, continue to proliferative phase during the first to months, plateau in growth during the second 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 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