Tufted angioma typically presents at birth (15% of cases) or within the first 5 years of life (50% of cases) as a violaceous plaque or nodule that may be painful. Rarely, it presents later in childhood or in early adulthood. There may be a slight male predominance.
After an initial period of growth, the tumor stabilizes and usually persists; in some patients, it may spontaneously, partially, or completely regress.
Three clinical patterns have been described: tufted angioma without complications, tufted angioma complicated with chronic coagulopathy without thrombocytopenia, and tufted angioma associated with Kasabach-Merritt phenomenon (KMP). KMP is a potentially life-threatening condition characterized by an enlarging vascular lesion, profound thrombocytopenia, consumptive coagulopathy, and often a microangiopathic hemolytic anemia.
D18.01 – Hemangioma of skin and subcutaneous tissue
254786000 – Tufted angioma of skin
Differential Diagnosis & Pitfalls
- Infantile hemangioma – Asymptomatic and soft; rapidly grows before a slow involution; no associated coagulopathy.
- Vascular malformation (eg, arteriovenous malformations, port-wine stain, Klippel-Trenaunay syndrome, acroangiodermatitis, lumbosacral infantile hemangioma, angioma serpiginosum, lymphangioma circumscriptum, megalencephaly-capillary malformation-polymicrogyria syndrome [MCAP])
- Infantile myofibromatosis
- Congenital dermatofibrosarcoma protuberans
- Kaposiform hemangioendothelioma
- Congenital hemangioma