Kaposiform hemangioendothelioma
ORPHA:2122· ICD-10 C49.9
Definition
A rare, locally aggressive, cutaneous or visceral vascular tumor characterized by either superficial lesions presenting as blue-purple infiltrated ill-defined plaques, or deep lesions usually manifesting with coagulation abnormality. They are associated in about 3 out of 4 of cases with potentially lethal thrombocytopenia and consumption coagulopathy (Kasabach-Merritt phenomenon; KMP).
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- All ages