Vascular birthmarks: Causes for concern, reasons for hope

National report - Vascular birthmarks in the pediatric population can create a wide array of questions and concerns for physicians and parents, says Ilona Frieden, M.D., of the department of dermatology, University of California, San Francisco.

"What is it? Is there a risk of extracutaneous associations? What is the prognosis? And what, if anything, needs to be done?"

The term "hemangioma" has been used to describe an array of pathogenically diverse diseases - lobular capillary; rapidly involuting congenital hemangioma (RICH); and non-involuting congenital hemangioma (NICH). Dr. Frieden discusses RICH, NICH and infantile hemangiomas (IH).

The rapidly involuting congenital hemangioma is a recently recognized birthmark that is fully formed at birth. This vascular birthmark exhibits no post-natal growth phase, but does exhibit a high vascular flow with or without shunting. Involution typically occurs by 18 months, and depending on the hemangioma's size and location, may leave residual skin changes.

Similar to RICH, but non-regressing, is the non-involuting congenital hemangioma (NICH). This birthmark typically is characterized by a flatter surface and is usually asymptomatic.

Infantile hemangiomas, commonly known as "strawberry" hemangiomas, are the most common vascular birthmark and afflict 4 percent to 5 percent of the pediatric population. Seen most often in Caucasian, pre-term females, this birthmark may be completely absent at birth, or present as a flat area of blanched, bruised or reddened skin. It is virtually never fully formed at birth. It has a unique immunohistochemical profile different from either RICH or NICH.

Red flags, rules of thumb

While infantile hemangiomas are a common problem, they present tremendous clinical variation.

"Dermatologists should concentrate on the three determinants of prognosis," Dr. Frieden says. "We need to look at the age at time of evaluation, location and morphology. And remember: The nature of the hemangioma tumor is dynamic.

"Birthmarks can be like Polaroid film," she adds. "They don't necessarily need to be present the day of birth. It's our job to be vigilant about their appearance, prognosis and treatment."

Timing is everything, according to Dr. Frieden. While absent or barely present at birth, the IH enters a rapid-growth phase during the first weeks to first months of life. The degree of growth is unpredictable; some grow slightly, while others increase dramatically. Despite this unpredictability, some rules are helpful in management:

After age of onset, the location of the birthmark is the second determinant of disease management. If the tumor is located in certain areas such as the periocular area or "beard" area, risk of extracutaneous medical morbidity (affecting the eye or airway, respectively) is significant. If the location is on the central face, nasal tip, perioral, ear or glabella, there is greater risk for disfigurement.

The morphology or location within the skin is the third determinant of disease, according to Dr. Frieden, as well as the pattern of skin involvement.

"Determining whether the involvement is segmental or localized is critical to determining prognosis and treatment," she says. Segmental hemangiomas are those involving a territory of skin, rather than appearing to arise from a single focus in the skin.