Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor, typically presenting in infancy as a distinctive cutaneous lesion with ill-defined borders. KHE can be confused with infantile hemangioma due to the age of presentation and presence of a vascular cutaneous lesion.

KHE tends to proliferate over several months followed by slow involution over many years.

KHE is an infiltrative tumor that has a tendency to cross tissue planes involving dermis, subcutis, fascia, muscle and bone.

T1 weighted imaging reveals characteristic, ill-defined hypointense soft tissue thickening involving multiple planes. T2 typically demonstrates hyperintense masses with reticular stranding in the subcutaneous fat.

Kasabach-Merritt phenomenon (KMP) is profound thrombocytopenia which results from intralesional platelet trapping. KMP occurs with KHE and tufted angioma but not infantile or congenital hemangioma.