Angioendotheliomatosis proliferans systematisata comprises two different entities; a malignant and benign (reactive) form. The more common malignant form with a fatal prognosis should be termed intravascular or angiotropic lymphoma and is a multifocal systemic disease, which affects especially the small and middle-sized blood vessels of the skin and central nervous system. Reactive angioendotheliomatosis is a benign disease, often associated with an infectious disease (predominantly with subacute bacterial endocarditis). We report a 38-year-old woman with a reactive angioendotheliomatosis and a 68-year-old man diagnosed as having the rare T-cell type of intravascular lymphoma.