Specialty oncology ICD-O M96803/{{{2}}} | ICD-10 D21 or C83.3 | |
Proliferating angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type.
The reactive involuting type is uncommon and occurs in patients with various diseases including subacute bacterial endocarditis and end-stage atherosclerotic disease. Patients present with various skin lesions and rashes - mostly commonly on the thighs. Treatment aimed at the underlying condition hastens the resolution of the lesions.
The malignant type is an intravascular lymphoma, usually of the diffuse B-cell type. It progresses rapidly through involvement of multiple body systems and mortality occurs in less than a year from the initial diagnosis. The average age of patients newly diagnosed is 55 years. Currently the causative mechanism is unknown. In a few cases treatment with palliative chemotherapy has been effective.
Classification
Proliferating angioendotheliomatosis may be divided into the following types: