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When to Worry, What to Do

Denise Walker Metry, MD; Adelaide A. Hebert, MD

Arch Dermatol. 2000;136:905-914.

Objectives  To discuss the current knowledge regarding complicated hemangiomas (cervicofacial, periorbital, lumbosacral, and parotid), including the associated syndromes of diffuse neonatal hemangiomatosis and PHACES (posterior fossa malformations, most commonly of the Dandy-Walker variant; hemangiomas [especially large, plaquelike, facial lesions]; arterial anomalies; cardiac anomalies and coarctation of the aorta; eye abnormalities; and sternal cleft and/or supraumbilical raphe). To discuss 2 newly recognized entities that may be a source of diagnostic confusion with the common hemangioma, the kaposiform hemangioendothelioma and tufted angioma. To discuss the risks and benefits of current treatment options, including the use of systemic corticosteroids and interferon in necessary situations.

Data Sources  The pertinent world literature was reviewed and incorporated into experience from our pediatric dermatology practice at the University of Texas Medical School at Houston.

Conclusions  The common hemangioma, kaposiform hemangioendothelioma, and tufted angioma, though benign histologically, may cause serious consequences for children. Dermatologists should be able to recognize unique clinical presentations of these lesions and obtain further diagnostic evaluation accordingly. Dermatologists should also be aware of available treatment options, including the use of systemic chemotherapy in life-threatening situations.

From the Departments of Dermatology (Drs Metry and Herbert) and Pediatrics (Dr Hebert), University of Texas Medical School at Houston. Dr Metry is now with the Department of Pediatric Dermatology, Baylor College of Medicine, Houston. The authors have no commercial, proprietary, or financial interest in the products or companies described in this article.

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