Malignant angioendotheliomatosis mimicking systemic necrotizing vasculitis
Kao, N.L.; Broy, S.; Tillawi, I.
Journal of Rheumatology 19(7): 1133-1135
1992
ISSN/ISBN: 0315-162X PMID: 1512773 Document Number: 398013
Malignant angioendotheliomatosis is a rare disease characterized by an intravascular proliferation of atypical mononuclear cells. Manifestations result from occlusion of small blood vessels. Multiple organ systems are involved and the clinical presentation resembles a systemic necrotizing vasculitis with skin and central nervous system most commonly involved. The clinical course is characterized by progressive organ failure with death usually within 2 years after presentation. Based on its assumed origin as an intravascular lymphoma, patients may respond to chemotherapy.