Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

About a third of patients with intravascular lymphoma (IVL) present to the neurologist with symptoms mimicking thromboembolic events. Diagnosis is difficult, and often made postmortem. As remission may be induced in almost half of patients with combination chemotherapy, early diagnosis of this rare disease is essential. We report two cases of IVL. A 62-year-old male presented with hyperacute myelopathy followed by cortical ischaemic events. The diagnosis was reached with frontal cortical and meningeal biopsy. A 56-year-old female had symptoms of transient ischaemic events, subacute dementia, weight loss and fever. As the disease progressed, she developed nephrotic syndrome and thrombocytopenia. Diagnosis was made postmortem. Our cases illustrate that IVL should be considered in the differential diagnosis of cerebral and systemic vasculitis and subacute bacterial endocarditis. Literature suggests IVL can also mimic Creutzfeld-Jakob disease and paraneoplastic encephalomyelitis.
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PMID:Neurological presentation of intravascular lymphoma: report of two cases and discussion of diagnostic challenges. 1612 73

We report the case of a 72-year-old woman with sporadic Creutzfeldt-Jakob's disease who presented a large purplish erythematous and edematous lesion, with subsequent bullous detachment on the anterior right thigh. The lesion rapidly evolved into an ulcer covered by a blackish necrotic eschar. Histological examination showed intense necrotizing leukocytoclastic vasculitis in the deep and middle dermis. Direct immunofluorescence revealed C3 and IgM deposits around vessels of the middle and deep dermis. The diagnosis of sporadic Creutzfeldt-Jacob's was confirmed post-mortem by immunoblotting on frozen brain tissue which showed pathologic proteinase-resistant prion-related protein isoform glycotype 2A. In the literature, only two cases of Creutzfeldt-Jakob's disease and cutaneous manifestations are reported.
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PMID:Skin vasculitis during Creutzfeldt-Jakob's disease. 1643 47