1/17. Large annular purpura and paraneoplastic purpura in a patient with sjogren's syndrome and cervical cancer.We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by sjogren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of sjogren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjogren's syndrome many also play a role in the development of allergic vasculitis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/17. adult Henoch-Schonlein purpura associated with malignancy.OBJECTIVE: Malignancies are among the well-established causes of vasculitis. We studied the association between adult Henoch-Schonlein purpura (HSP) and malignant neoplasms. methods: We retrospectively reviewed 14 cases of adult HSP diagnosed during a 6-year period and found a malignant neoplasm in four. Fifteen reports of adult HSP with malignant disease were identified in the literature. These 19 cases were compared with 158 adults who had HSP but no malignancy and who where reported in the literature. RESULTS: Most (63%) of the malignant neoplasms associated with adult HSP were solid tumors: lung, n = 6; prostate, n = 2; breast, n = 1; renal, n = 1; stomach, n = 1; and small bowel, n = 1. Hematologic malignancies (37%) included non-Hodgkin lymphoma, n = 2; hodgkin disease, n = 2; IgA multiple myeloma, n = 1; myeloproliferative disease, n = 1; and myelodysplastic syndrome, n = 1. patients with malignancy were older (median age, 59 years), were more likely to be male (M/F = 8.5), had joint involvement more frequently (95%), and had a lower frequency of prior acute infection than those without malignancy. A typical paraneoplastic course was documented in only two cases. CONCLUSIONS: Epidemiological studies are needed to determine the association between adult HSP and malignancy. In practice, it may be wise to suspect a malignancy in men older than 40 years of age who develop HSP in the absence of a precipitating factor. Pathogenic hypotheses involve tumor antigens or abnormal IgA production leading to immune complex formation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/17. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl.CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpastures syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 %, platelets = 260,000, white blood cells = 8300, 74 % segmented, 4 % eosinophils, 19 % lymphocytes and 3 % monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/17. Recurrent post-infective Henoch-Schonlein syndrome: a genetic influence related to HLA B35?The clinical features, laboratory findings, infection stimuli and HLA phenotypes of five young adults with recurrent episodes of Henoch-Schonlein syndrome (HSS) are reported. We define recurrences as the reappearance of the characteristic purpuric rash and associated symptoms more than 8 weeks after the onset of the original episode of purpura. All patients had documented evidence of a bacterial or viral infection before one or more of the relapses, the periods between which varied from 1 to 13 years. Four of our five patients possessed the HLA B35 phenotype and two of these four patients were also HLA B18 antigen positive. Those with the HLA B35 haplotype had recurrent episodes of purpura with nephritis triggered by minor pharyngeal (viral or bacterial) infections. The HLA B35 haplotype has a frequency of 4% in the indigenous Scottish population and has previously been linked with single episodes of the Henoch-Schonlein syndrome with nephritis in German, Slavic and French patients. It has not previously been related to recurrent episodes of the syndrome. We postulate that patients who are HLA B35 positive may be genetically more susceptible to recurrent episodes of HSS with nephritis, stimulated by a heterogeneous group of infective stimuli and resulting in a protracted illness with significant renal involvement.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/17. IgA nephritis in HIV-positive patients: a new HIV-associated nephropathy?Four HIV-positive patients were shown to have IgA-associated nephritis on biopsy, including one with anaphylactoid purpura. Three were homosexuals, while the fourth acquired the infection from his mother. All had hematuria, a variable degree of proteinuria and renal disease with a benign course. Serologic studies showed elevated levels of IgA as well as IgA immune complexes and rheumatoid factor. IgA antibodies to multiple hiv antigens were detected by Western blot. Pathologic studies showed tubuloreticular inclusions in endothelial cells and nuclear bodies in interstitial cells in all cases. hiv antigens were not detected in kidney biopsies by monoclonal antibodies nor was HIV viral genome demonstrated by in situ hybridization. The possibility that this represents a unique type of IgA-associated HIV nephropathy is discussed.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/17. purpura fulminans due to staphylococcus aureus.BACKGROUND: purpura fulminans is an acute illness commonly associated with meningococcemia or invasive streptococcal disease, and it is typically characterized by disseminated intravascular coagulation (DIC) and purpuric skin lesions. In this article, we report the first 5 cases (to our knowledge) of purpura fulminans directly associated with staphylococcus aureus strains that produce high levels of the superantigens toxic shock syndrome toxin-1 (TSST-1), staphylococcal enterotoxin serotype B (SEB), or staphylococcal enterotoxin serotype C (SEC). methods: Cases were identified in the Minneapolis-St. Paul, minnesota, metropolitan area during 2000-2004. S. aureus infection was diagnosed on the basis of culture results, and susceptibility to methicillin was determined. The ability of the isolated organisms to produce TSST-1, SEB, SEC, and Panton-Valentine leukocidin (PVL) was determined. TSST-1, SEB, and SEC levels were also quantified after in vitro growth of the organisms. RESULTS: In 3 of the 5 cases, the infecting S. aureus strain was isolated from the blood cultures. In 2 of the 5 cases, the infecting S. aureus strain was isolated only from the respiratory tract, indicating that purpura fulminans and toxic shock syndrome resulted from exotoxin and/or other host factors, rather than septicemia. One of these latter 2 patients also had necrotizing pneumonia, and the isolated S. aureus was a methicillin-resistant strain that produced both SEC and PVL. Only 2 of the 5 patients survived, and 1 of the survivors received activated protein c. CONCLUSIONS: Staphylococcal purpura fulminans may be a newly emerging illness associated with superantigen production. Medical practitioners should be aware of this illness.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/17. Interest of postmortem-collected specimens in the diagnosis of fulminant meningococcal sepsis.We reported the case of a child who died of purpura fulminans. The diagnosis of neisseria meningitidis serogroup C could be assessed using postmortem specimens collected up to 10 h after death. We were able to identify the bacteria by culture and/or PCR on samples without having autopsy performed. Soluble antigens were also detected in serum.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/17. Henoch-Schonlein purpura in a child following varicella.Henoch-Schonlein purpura (HSP) is one of the most common vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, arthritis, renal and gastrointestinal system (GIS) involvement. HSP is usually triggered by an antigenic stimulus including infectious agents, drugs, cold, insect bite or food. HSP is rarely triggered by Varicella zoster infection. We herein presented a case with HSP following varicella.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/17. Henoch-Schonlein purpura and Crohn's disease in a family.A 16-year-old female who underwent an appendicectomy had terminal segmental ileitis, and developed Henoch-Schonlein purpura (HSP) a few days later. Her brother had suffered from post-infection HSP, while her mother has suffered from Crohn's disease. Human leukocyte antigen (HLA) typing in the patient disclosed the DRB1*11 allele, which has been reported to be associated with HSP, but the brother proved negative, suggesting that this allele was irrelevant to the HSP pathogenesis. The patient and the other relatives did not disclose HLA DRB1*01, which is the only class II phenotype reported to be associated with both diseases. While this case report lends support to the idea that the earlier observation of concomitant Crohn's disease and HSP in the same patients is no chance association, it suggests that if the two pathological conditions share a common genetic background, this does not seem to be related to class II HLA phenotypes. Other, as yet unknown genes could be involved.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/17. Henoch-Schonlein nephritis.Henoch-Schonlein nephritis now appears to be an immunologically mediated disease, common in children but rare in adults, and initiated by unknown antigen or antigens. We have reviewed this form of nephritis with particular attention to immunological changes, occurrence in adults and prognosis. Assessment and treatment are made difficult by varying diagnostic criteria, especially in adults, and lack of controlled data.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
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