Tufted angiomas (TA) are rare benign vascular tumors of unknown pathogenesis. Most appear during childhood; approximately 25 percent are congenital and 50 percent appear in the first year of life. According to the literature, TA that are present at birth or in the first year of life have a greater tendency to spontaneously regress than do those that appear later in life. Their clinical presentation is non-specific and characterized by bluish-erythmatous plaques or nodules. The differential diagnosis includes infantile hemangiomas, congenital hemangiomas, kaposiform hemangioendothelioma and vascular malformations. Tufted angiomas have a characteristic histology consisting of a proliferation of endothelial cells forming lobules with the typical "shotgun" distribution. We report a case of congenital TA and review the cases of congenital TA described to date in the literature in order to highlight the different characteristics of congenital and acquired TA.

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