Angioendotheliomatosis proliferans systematisata comprises two different en
tities; a malignant and benign (reactive) form. The more common malignant f
orm with a fatal prognosis should be termed intravascular or angiotropic ly
mphoma and is a multifocal systemic disease, which affects especially the s
mall 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.