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1: J Rheumatol. 1992 Jul;19(7):1133-5.Links

Malignant angioendotheliomatosis mimicking systemic necrotizing vasculitis.

Kao NL, Broy S, Tillawi I.

Lutheran General Hospital, Division of Medicine, Park Ridge, IL 60068.

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.

PMID: 1512773 [PubMed - indexed for MEDLINE]