Tufted angioma
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Synopsis
Tufted angioma (TA), previously known as angioblastoma of Nakagawa, is a rare, benign, vascular tumor that is thought to be on the same clinical spectrum as kaposiform hemangioendothelioma (KHE). The name describes the presence of "tufts" of capillaries in the dermis as seen on histopathological sections.
TA typically presents at birth (15%-25% of cases) or within the first 5 years of life (50% of cases) as a red-violaceous plaque or nodule. Rarely, TAs arise in adulthood. There is no consistent sex or racial / ethnic predilection.
A TA will generally grow slowly over several months to up to a decade before stabilizing in size, and partial or complete regression may occur. TAs are initially painless, but they can become painful in a significant proportion of patients.
Three clinical patterns have been described: TA without complications, TA complicated with chronic coagulopathy without thrombocytopenia, and TA associated with Kasabach-Merritt phenomenon (KMP). The mortality rate from KMP is high, from 10%-30%.
TA typically presents at birth (15%-25% of cases) or within the first 5 years of life (50% of cases) as a red-violaceous plaque or nodule. Rarely, TAs arise in adulthood. There is no consistent sex or racial / ethnic predilection.
A TA will generally grow slowly over several months to up to a decade before stabilizing in size, and partial or complete regression may occur. TAs are initially painless, but they can become painful in a significant proportion of patients.
Three clinical patterns have been described: TA without complications, TA complicated with chronic coagulopathy without thrombocytopenia, and TA associated with Kasabach-Merritt phenomenon (KMP). The mortality rate from KMP is high, from 10%-30%.
Codes
ICD10CM:
D18.01 – Hemangioma of skin and subcutaneous tissue
SNOMEDCT:
254786000 – Tufted angioma of skin
D18.01 – Hemangioma of skin and subcutaneous tissue
SNOMEDCT:
254786000 – Tufted angioma of skin
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Last Reviewed:03/25/2026
Last Updated:03/25/2026
Last Updated:03/25/2026
Tufted angioma
