Tufted angioma

Tufted angioma (also known as angioblastoma of Nakagawa or progressive capillary hemangioma) is a rare benign vascular tumor that primarily affects the skin. It is characterized by the presence of slow-growing, firm, red to purple-brown plaques or nodules that most commonly appear on the neck, upper trunk, and shoulders, though they can occur anywhere on the body. The lesions are composed of small tufts or lobules of capillaries scattered throughout the dermis and subcutaneous tissue, giving the tumor its characteristic histological appearance. Tufted angiomas typically develop during infancy or early childhood, though they can occasionally present at birth or in adulthood. A significant clinical concern with tufted angioma is its association with Kasabach-Merritt phenomenon (KMP), a potentially life-threatening condition characterized by profound thrombocytopenia, consumptive coagulopathy, and microangiopathic hemolytic anemia caused by platelet trapping within the vascular tumor. Not all tufted angiomas develop KMP, but when it occurs, it requires urgent medical intervention. Affected individuals may experience pain, tenderness, hyperhidrosis (excessive sweating), or hypertrichosis (increased hair growth) over the lesion. Treatment of tufted angioma depends on the size, location, and presence of complications. Small, asymptomatic lesions may be monitored without intervention, as some cases show partial spontaneous regression over time. For symptomatic or complicated cases, treatment options include surgical excision when feasible, systemic corticosteroids, vincristine, sirolimus (an mTOR inhibitor that has shown promising results), aspirin, ticlopidine, and interferon-alpha. In cases complicated by Kasabach-Merritt phenomenon, aggressive medical management with sirolimus or vincristine is often employed. Complete surgical removal can be curative but is not always possible due to the diffuse nature of some lesions.

Common questions about Tufted angioma