Classification

Hemangiomas may be classified by their depth in the skin, number or location in the body. Many hemangiomas consist entirely of a cutaneous component and will present clinically as a bright red non-compressible papule, nodule or plaque when fully developed. These cutaneous or superficial hemangiomas represent 50-60% of all hemangiomas and can resemble a strawberry when mature. (Figure1a) Hemangiomas can develop subcutaneously without a cutaneous component. When this occurs the hemangioma is described as a subcutaneous or deep hemangioma. (Figure 1b) Subcutaneous lesions may be located so deep within the skin that they appear as flesh colored masses. As a hemangioma becomes located closer to the skin surface, it will appear as a nodule or mass with a bluish coloration. Occasionally dilated veins or telangiectasias are seen overlying the surface of a subcutaneous hemangioma. When a cutaneous (red) component is seen with an underlying, deeper induration (subcutaneous component) then the lesion is classified as a compound or mixed hemangioma. (Figure 1c) The terms "cavernous or capillary hemangioma" are antiquated terms that are not useful terms for describing hemangiomas. Field hemangiomas are those hemangiomas that cover a large area and begin as multiple erythematous macules that rapidly enlarge and coalesce (Figure 1d). These lesions are often mistaken for portwine stains until they begin to proliferate. Visceral hemangiomas are those that occur in deeper organ systems such as the liver, colon or brain.

Hemangiomas can also be classified by number. The majority of patients present with a single lesion, however 20% of patients will have more than one hemangioma.¹² The presence of three lesions is uncommon and the presence of four or more hemangiomas is rare. When infants present with 3 or more hemangiomas then they are referred to as having multiple hemangiomas (Figure 2). The presence of multiple cutaneous hemangiomas greatly enhances the chances that the child could have visceral hemangiomas. It is uncommon for an infant with visceral hemangiomas not to have cutaneous involvement.^8,14

In the past, children with multiple skin hemangiomas without visceral involvement were said to have "benign neonatal hemangiomatosis" and those with visceral involvement were said to have "diffuse  neonatal hemangiomatosis".^15-22 We prefer the terminology of multiple hemangiomas with or without visceral involvement. It is important to identify visceral hemangiomas in patients having multiple hemangiomas. The liver is the most common location for visceral lesions.^14,16 Hepatic hemangiomas occur 16% of the time followed by the brain (13%), intestines (13%), lungs (13%) and tongue (11%).¹⁶ Hepatic hemangiomas are more closely associated with high output congestive heart failure, thrombocytopenia and hemorrhage, ultimate causes of morbidity in these patients.¹⁴ Accordingly, liver involvement has been associated with a poorer prognosis.

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