A 3-year-old boy exhibited a congenital, erythematous-violaceous plaque with discreet hypertrichosis on his right thigh (Fig. 1A). Histopathological examination revealed the presence of fibrosis and lobules formed by capillaries on both the superficial and deep dermis, distributed in a “shotgun” pattern and surrounded by crescent moon-shaped vessels (Fig. 1B, HE ×10).

Figure 1

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Since the first months of life, the patient had recurrent episodes of pain, increased local size and temperature, sometimes associated with fever and elevated acute phase reactants. Although the blood test results did not reveal the presence of thrombocytopenia or fibrinogen changes, they showed sustained elevation of D-dimer and hypogammaglobulinemia. The patient was treated with acetylsalicylic acid, ticlopidine, topical sirolimus, oral sirolimus, heparin, and oral corticosteroid cycles. Due to persistent pain and difficulty walking, surgical treatment was eventually performed.

Tufted angioma (TA) and kaposiform hemangioendothelioma are rare vascular tumors that fall within the same spectrum. In more than half of the cases, TA tends to resolve spontaneously. There are times, however, when it may present with pain and functional impairment, and even become complicated with the Kasabach-Merritt phenomenon, which means that patient monitoring is essential.