Lawrence Eichenfield, M.D.

Tremendous health risks are associated with hemangiomas in neonates, requiring dermatologists to rapidly diagnose and manage these challenging conditions. Offering recommendations for care was Lawrence Eichenfield, M.D.,chief of pediatric and adolescent dermatology, Rady Children's Hospital, San Diego, and professor of clinical pediatrics and medicine (dermatology) at the University of California, San Diego, School of Medicine, during his plenary session presentation, "Neonatal Dermatology: Recent Developments."

"Due to a combination of scientific advances and clinical observations, we've changed our understanding and management of hemangiomas in infants," Dr. Eichenfield said. "In addition, due to advances in neonatal care allowing the support of very premature infants and other factors, a variety of opportunistic skin infections are increasingly common."

He credited the Hemangioma of Infancy Study Group and others with adding to the body of knowledge about the pathogenesis, risk factors and the natural course of progression for these birthmarks.

"Hemangiomas are lesions commonly present in 4 percent to 10 percent of newborns," Dr. Eichenfield said. "Hemangiomas in infancy can be either localized, multiple or segmental.

Segmental hemangiomas and PHACE syndrome
In particular, segmental hemangiomas on the face are associated with PHACE Syndrome, which is associated with significant systemic abnormalities. PHACE is an acronym that describes the syndrome with the following features: posterior fossa brain malformations, large facial hemangiomas, arterial anomalies, cardiac anomalies and aortic coarctation, and eye abnormalities. Studies have now expanded the system anomalies associated with PHACE and established how common these problems can be. In a study of 108 infants with large facial hemangiomas, 31 percent had PHACE Syndrome. Of those with PHACE Syndrome, 91 percent had cerebrovasculature abnormalities and 67 percent had cardiac anomalies.

"The diagnostic criteria now for PHACE Syndrome will set a new standard of care for addressing facial hemangiomas in children," Dr. Eichenfield said. "Any child with a significant facial hemangioma should now undergo a standard work-up for PHACE Syndrome, which will include MRIs of the head and neck, echocardiography and ophthalmologic examination. Certain regions of the face do carry a higher risk for abnormalities."

New etiology information available
In addition to diagnostic advances, much more information is now available about the etiology of hemangiomas, he noted. Research indicates that vascular endothelial growth factor (VEGF) is important as a regulator of endothelial cell growth for feeding hemangiomas and that hemangiomas may reflect an imbalance of two epidermal growth factor receptors (EGFR-1 and EGFR-2).

"Just as exciting as the changing diagnostic and pathogenetic aspects of hemangiomas is an incredible breakthrough in the use of propranolol, a beta blocker often used for hypertension or cardiac strain," Dr. Eichenfield said. "In many patients, propranolol results in minimizing growth and causing rapid shrinkage of hemangiomas, and can be used as either a single therapy or as multi-modal therapy in combination with other agents."

Uses of propranolol worldwide have shown it appropriate as a first-line treatment for significant hemangiomas affecting function or causing deformation, blocking the airway or presenting in organs other than the skin, such as the brain and liver, he said. Nevertheless, this off-label use of propranolol for hemangiomas is not without its problems, which can include cardiovascular collapse and/or seizures, with or without hypoglycemia.

"We have both excitement about this tremendous therapy, but also significant caution as we try to figure out its placement, relative efficacy, appropriate timing of use and safety as compared to its other benefits for treating hemangiomas," Dr. Eichenfield said.

Neonates and opportunistic skin infection
The second part of his lecture addressed the increasing number of opportunistic skin infections presenting in neonates over the past decade.

"These infections are especially common in premature infants whose skin development is incomplete at the time of delivery and whose skin stratum corneum function is compromised over several weeks," Dr. Eichenfield said. "This immature skin creates a risk of both fluid and electrolyte loss, temperature dysregulation, increased uptake of potentially toxic agents that come into contact with the skin and increased vulnerability to injury and infection."

Both candida and fungal infections have been known to cause significant morbidity and mortality in neonates, he said. Clinical presentation of candida and fungal infections can include papules, pustules, crusts, ecchymosis, erosions, and ulcerations.

"These infections are ‘outside-in' ones where the yeast or fungal species is initially invading the cutaneous surface," Dr. Eichenfield said. "With prompt diagnosis and initiation of therapy, it can be easily managed, but with delayed diagnosis, these infections can become blood-borne and seed major organs, including brain, lung, and liver, and will result in a significant mortality."

He further recommended that dermatologists watch carefully for various presentations in ill neonates, especially premature neonates, and consider opportunistic infection in the differential diagnosis of skin lesions. This is certainly the time for liberal skin biopsy for both microscopic examination and culture, which could be a life-saving intervention.

"There have been tremendous advances in neonatal dermatology, and the field is developing very rapidly," Dr. Eichenfield said. "It is very important for us to keep abreast of these changes so that they can bring the best care to neonates and infants."