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Tufted angioma
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Tufted angioma

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Contributors: Erin X. Wei MD, Shilpa S. Sawardekar MD, Susan Burgin MD
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Synopsis

Tufted angioma : Trunk, Tumor, Vascular plaque, Painful skin lesion
Tufted angioma, previously known as angioblastoma of Nakagawa, is a rare vascular tumor. The name describes the histologic appearance of "tufted" capillary infiltration of the dermis admixed with dilated lymphatic vessels. It is theorized to have a lymphatic origin.

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.

Codes

ICD10CM:
D18.01 – Hemangioma of skin and subcutaneous tissue

SNOMEDCT:
254786000 – Tufted angioma of skin

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Last Reviewed:08/11/2021
Last Updated:01/25/2022
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Tufted angioma
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A medical illustration showing key findings of Tufted angioma : Trunk, Tumor, Vascular plaque, Painful skin lesion
Clinical image of Tufted angioma - imageId=2384753. Click to open in gallery. caption: 'A faint pink plaque with an uneven surface on the posterior thigh.'
A faint pink plaque with an uneven surface on the posterior thigh.

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