1/10. A case of melena caused by a hepatic aneurysm ruptured into the intrahepatic bile duct in a patient with allergic granulomatous angiitis.A 46 year old woman was admitted to our institute in June, 1987 with an attack of asthma, as well as remittent fever and leukocytosis accompanied by hypereosinophilia. She was found to have melena from an unknown source upon gastrointestinal examination. Four low-density areas were found in the liver on computed tomography and one of the intrahepatic foci formed a large extrahepatic abscess communicating with the intrahepatic duct on tubography. Resection of the four hepatic segments, including the large abscess, and cholecystectomy were performed. Healed necrotizing arteritis was histopathologically observed in the resected liver specimen, with the four low-density areas on CT scan having all been necrotic foci. One of them formed an intrahepatic biliary fistula and rupture of a hepatic aneurysm into a biliary duct was found to be the cause of melena. Although eosinophil infiltration and extravascular granuloma were not observed, a diagnosis of allergic granulomatous angiitis was made from the characteristic clinical course, systemic vasculitis and peripheral blood eosinophilia. To the best of our knowledge, this is the first report of intrahepatic duct perforation most probably being caused by hepatic aneurysm rupture in a patient with allergic granulomatous angiitis.- - - - - - - - - - ranking = 1keywords = allergic granulomatous angiitis, granulomatous angiitis, angiitis, vasculitis (Clic here for more details about this article) |
2/10. Gastrointestinal manifestations of Henoch-Schonlein purpura.We report a case of the intestinal lesion in Henoch-Schonlein purpura, presented with an acute abdomen in a 4 year old boy. Five days after sudden colicky abdominal pain, skin purpura and painful joint swelling developed. These manifestations were associated with abdominal distension, hematemesis, hematochezia and hematuria. Exploratory laparotomy revealed a marked bowel distension with edema and patchy dark reddish discoloration of the jejunum and ileum. These patchy areas showed transmural hemorrhage and necrosis associated with characteristic leukocytoclastic vasculitis in and around the hemorrhagic lesions. These vasculitis was thought to be related to Henoch-Schonlein purpura.- - - - - - - - - - ranking = 9.8831342157269E-6keywords = vasculitis (Clic here for more details about this article) |
3/10. Leukocytoclastic vasculitis of the colon.Two cases of young patients with an acute abdomen caused by a necrotizing angiitis of the cecum and ascending colon are reported. The absence of any systemic disease and a history of medical treatment in both cases led to the diagnosis of hypersensitivity vasculitis by a serum sickness-like reaction. An extensive review of the literature is also presented.- - - - - - - - - - ranking = 0.00040668316991559keywords = angiitis, vasculitis (Clic here for more details about this article) |
4/10. Acute febrile neutrophilic dermatosis: Sweet's syndrome.A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) is described in a patient with chronic myelogenous leukemia, with a review of all published reports in the English literature. The disease is characterized by an acute febrile illness with painful plaques involving the extremities, face, and neck, and responds dramatically to corticosteroids with occasional recurrences. Histologically, the skin biopsy shows a dense dermal infiltrate of polymorphonuclear leukocytes without evidence of vasculitis.- - - - - - - - - - ranking = 4.9415671078634E-6keywords = vasculitis (Clic here for more details about this article) |
5/10. Multiple cholesterol emboli syndrome simulating systemic necrotizing vasculitis.The multiple cholesterol emboli syndrome associated with severe atherosclerotic vascular disease may clinically simulate systemic necrotizing vasculitis. Widespread visceral infarctions in conjunction with potential leukocytosis, eosinophilia, thrombocytopenia, hypocomplementemia and an elevated sedimentation rate may be present. It frequently occurs after intraarterial procedures such as cardiac catheterization and aortography. diagnosis is made by biopsy of involved tissue. It is essential to establish a proper diagnosis to avoid inappropriate use of steroid and cytotoxic treatment measures.- - - - - - - - - - ranking = 2.4707835539317E-5keywords = vasculitis (Clic here for more details about this article) |
6/10. Veno-occlusive disease of the liver in a patient with allergic granulomatous angiitis.A 45-yr-old woman, with asthma and skin erythema, was diagnosed as having allergic granulomatous angiitis. leukocytosis accompanied by hypereosinophilia and characteristic histological findings of skin lesions also were present. Upon laparoscopy and liver biopsy, the liver showed a congestive appearance and, histologically, showed thickening of the central vein walls, as well as narrowing of the lumens with collagenous fibers, and pericentral hemorrhage with hepatocyte degeneration. A diagnosis of veno-occlusive disease of the liver was made. This is the first report on a case of veno-occlusive disease of the liver occurring in association with allergic granulomatous angiitis.- - - - - - - - - - ranking = 0.99999505843289keywords = allergic granulomatous angiitis, granulomatous angiitis, angiitis (Clic here for more details about this article) |
7/10. Pustular vasculitis of the hands.BACKGROUND: Several patients were observed with a peculiar cutaneous eruption limited to the dorsa of the hands and fingers. Clinically the lesions had some resemblance to those seen in Sweet's syndrome, but biopsy specimens showed severe leukocytoclastic vasculitis. OBJECTIVE: Our purpose was to characterize this eruption clinically and histologically and compare it with previously described diseases. methods: Six patients observed since 1977 are described. skin biopsy specimens were obtained. RESULTS: In six women (age, 41 to 79 years) a symmetric eruption of papules and plaques limited to the dorsa of the radial sides of the hands and first three digits developed. The lesions resembled those of Sweet's syndrome and were associated with fever, sterile culture, blood neutrophil leukocytosis, nonresponse to antibiotic therapy, and rapid response to prednisone. biopsy specimens showed a severe leukocytoclastic vasculitis. CONCLUSION: These patients appear to have a distinct entity that we have termed pustular vasculitis of the hands.- - - - - - - - - - ranking = 3.4590969755044E-5keywords = vasculitis (Clic here for more details about this article) |
8/10. Acute febrile toxic reaction in patients with refractory rheumatoid arthritis who are receiving combined therapy with methotrexate and azathioprine.OBJECTIVE: To assess the frequency and clinical features of an acute febrile toxic reaction (AFTR) in patients with refractory rheumatoid arthritis (RA) receiving combined therapy with methotrexate (MTX) and azathioprine (AZA). methods: A cohort of 43 RA patients being treated with MTX/AZA combination therapy were studied. In all of them, RA had been refractory to single-therapy disease-modifying antirheumatic drugs. We analyzed the frequency and clinical features of AFTR, which consisted mainly of the development of fever, leukocytosis, and cutaneous leukocytoclastic vasculitis when AZA was added to the MTX regimen. RESULTS: Four of the 43 patients (9.3%) who had been receiving long-term, well-tolerated treatment with MTX (mean /- SD 375.5 /- 159.5 days, range 227-561 days) developed AFTR shortly (mean /- SD 25.7 /- 13.6 days, range 17-46 days) after the addition of AZA to the regimen. The AFTR resolved rapidly (3 /- 1.4 days) after discontinuation of AZA and MTX. In 2 cases, rechallenge with AZA and MTX was linked to a new flare of AFTR. CONCLUSION: The knowledge of this side effect is particularly important because it mimics a severe infectious complication related to immunosuppressive therapy, and because rechallenge can produce severe toxicity. Most of the new combined therapies for RA do not seem to be more toxic than single-drug treatment. Nevertheless, clinicians should be aware of a possible increase in side effects due to drug interactions or some other unidentified mechanism.- - - - - - - - - - ranking = 4.9415671078634E-6keywords = vasculitis (Clic here for more details about this article) |
9/10. Profound cerebrospinal fluid pleocytosis and Froin's syndrome secondary to widespread necrotizing vasculitis in an hiv-positive patient with varicella zoster virus encephalomyelitis.Demonstration of the direct involvement of cranial blood vessels by varicella zoster virus (VZV) is facilitated by immunohistochemistry (IHC), in situ hybridization (ISH) and polymerase chain reaction (PCR) techniques. The extent to which an inflammatory vasculitis serves as the pathogenic mechanism for VZV encephalomyelitis (VZVE) is still, however, debated. Most VZVE patients are immunocompromised and show little inflammation, either pre-mortem in cerebrospinal fluid (CSF) or at autopsy. We describe an hiv-positive patient with a moderately depressed CD4 count (304) who presented with massively elevated CSF protein (1800 mg/dl), bloody CSF and pleocytosis (1300 white blood cells (WBC)/mm3). His CSF was positive for VZV dna by PCR. He was treated with acyclovir and foscarnet, but died. At autopsy, an unusually widespread, inflammatory, transmural vasculitis caused by VZV affected meningeal vessels at virtually all brain stem and spinal cord levels, causing multiple subpial hemorrhages and necrosis. Virus dna in multiple areas of brain, brainstem and spinal cord was readily revealed by PCR, but not by the presence of viral inclusions, IHC or ISH. This case, with a clinically confusing presentation for VZVE, illustrates the extensive, albeit infrequent, degree of necrotizing vasculitis and CSF abnormalities that VZV is capable of producing. Antiviral therapy may have inhibited VZV genome replication and subsequent antigen production, resulting in negative ISH and IHC studies, but generated increased VZV genomic fragments that were detectable by the more sensitive PCR technique.- - - - - - - - - - ranking = 3.4590969755044E-5keywords = vasculitis (Clic here for more details about this article) |
10/10. De novo status epilepticus as the presenting sign of neurosyphilis.Although the incidence of seizures in neurosyphilis ranges from 14 to 60%, status epilepticus (SE) as a presenting complaint of neurosyphilis is definitely rare. A 44-year-old human immunodeficiency virus (hiv)-negative man with no history of epilepsy suddenly presented with acute mental confusion and was diagnosed as having a de novo complex partial nonconvulsive SE. cerebrospinal fluid (CSF) findings, neuroimaging, and clinical course indicated that SE was the presenting symptom of an undiagnosed syphilitic meningovasculitis. The case is presented with a review of previous reports to emphasize the differential features and to underscore the importance of considering neurosyphilis among the possible causes of de novo SE.- - - - - - - - - - ranking = 4.9415671078634E-6keywords = vasculitis (Clic here for more details about this article) |
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