Malignant angioendotheliomatosis mimicking systemic necrotizing vasculitis.
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.
Kao
N L
NL
Lutheran General Hospital, Division of Medicine, Park Ridge, IL 60068.
Broy
S
S
Tillawi
I
I
D003937:Diagnosis, Differential; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D014657:Vasculitis / Q000175:diagnosis* / Q000473:pathology