Cases reported "Disease Progression"

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1/99. Late development of antidesmoglein 1 antibodies in pemphigus vulgaris: correlation with disease progression.

    The coexistence of antidesmoglein 3 (Dsg3) and antidesmoglein 1 (Dsg1) autoantibodies is well described in patients with pemphigus vulgaris (PV); however, there is little evidence of sequential development of these two autoantibodies. Autoantibody responses to Dsg3 and Dsg1 were studied in seven PV patients over time by enzyme-linked immunosorbent assay, using baculovirus expressed recombinant fusion proteins. All patients had anti-Dsg3 IgG antibodies at presentation. Two patients developed anti-Dsg1 later in the course of the disease. The transition in autoantibody profile was associated with disease progression to generalized PV involving mucous membranes and skin in both patients; one patient initially presented with a predominantly mucosal phenotype, the other with herpetiform pemphigus-like features. These findings demonstrate that there is an extension of autoimmune response from anti-Dsg3 only to both anti-Dsg3 and anti-Dsg1 in some patients, which is associated with an alteration in clinical expression in PV.
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ranking = 1
keywords = membrane, mucosa
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2/99. Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa.

    We describe two patients with acquired tufted angioma, a unique vascular lesion not previously reported in the oral mucosa. In one patient, the lesion manifested as a purple-red papule and, in the other, as a blue submucosal swelling. Both lesions were non-painful and neither was associated with a history of trauma. The histopathological features consisted of scattered, irregularly shaped tufts, primarily composed of poorly formed capillary spaces and slit-like vascular channels. Capillary spaces were often closely packed, producing solid areas which stained for smooth muscle actin. Staining for factor viii-related antigen was positive only within endothelial cells lining well-formed vascular channels. Both lesions were treated by excision; short-term follow-up of one patient revealed no evidence of recurrence. Similarities between this and other vascular processes may have resulted in misdiagnosis of this lesion in the past. The clinical significance of acquired tufted angioma in the oral mucosa is not known.
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ranking = 3.4322644081199
keywords = mucosa
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3/99. campylobacter jejuni-induced severe colitis--a rare cause of toxic megacolon.

    The development of toxic megacolon as a sequel of infectious colitis is rare. We have observed the very rare case of a campylobacter jejuni-induced toxic megacolon. A 28-year-old man was admitted with severe enterocolitis and appearance of blood in stools. He had been treated with loperamide without success. Two days after admission stool cultures revealed campylobacter jejuni and then an oral antibiotic therapy was started. On the fifth day clinical performance deteriorated again with development of toxic megacolon and consecutive subtotal colectomy. Rectoscopy before discharge after 13 days showed a normal mucosa. The unusual course with first improvement and then rapid deterioration despite adequate therapy was observed in 4 other cases, which may also be a hint of ensuing megacolon. Even in usually harmless enterocolitis like campylobacter infection, predisposing factors such as loperamide are known to precipitate toxic megacolon and should be considered in clinical practice.
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ranking = 0.49032348687428
keywords = mucosa
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4/99. helicobacter pylori, gastric MALT lymphoma, and adenocarcinoma of the stomach.

    The discovery of helicobacter pylori and its relationship to upper gastrointestinal tract diseases has emphasized the significance of infectious pathogens in clinical disease. Severe manifestations of H. pylori-associated diseases include gastric adenocarcinoma and the recently described gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Ongoing worldwide investigations of the interactions of H. pylori and the host response are rapidly clarifying the role of this bacterium in multiple gastrointestinal diseases. This review will address diagnosis, management, and follow-up of the patient presenting with gastric MALT lymphoma, including a discussion of the issues related to premalignant lesions associated with gastric adenocarcinoma. Prospective trials and long-term follow-up studies are in progress and will guide appropriate management of these diseases.
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ranking = 0.49032348687428
keywords = mucosa
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5/99. A case of infantile neuroblastoma with intramucosal metastasis in a paranasal sinus.

    An 8-year-old boy with neuroblastoma of the right adrenal gland is reported. His initial treatment included chemotherapy and surgery, with complete response (CR) being achieved at the initial site. A metastatic lesion was found in the right maxillary sinus 32 months after his initial treatment. A mass in the right soft palate was detected and was clinically suspected of being a metastasis. The results of biopsy were negative and the differential diagnosis from the imaging studies of CT included odontogenic disease, fungal infection, paranasal sinus cyst or hematoma, and benign tumors. Open transantral biopsy was done under general anesthesia, revealing severe inflammation in the right maxillary sinus as well as bone erosion. The histopathological diagnosis was metastatic neuroblastoma from the adrenal lesion. The local field was irradiated with 20 Gy of linear accelerator (linac) radiation, then the local field was eradicated. Extensive skeletal metastases were subsequently found by bone scintigraphy. Despite further treatment his general condition deteriorated rapidly and he died 24 months after starting treatment. We review the previous reports and discuss metastasis to the sinuses.
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ranking = 1.9612939474971
keywords = mucosa
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6/99. Do B cells influence disease progression in chronic synovitis? Lessons from primary hypogammaglobulinaemia.

    We describe a 62-yr-old male patient with primary hypogammaglobulinaemia (PH) who fulfilled the 1987 American Rheumatism association/American College of rheumatology revised diagnostic criteria for rheumatoid arthritis (RA) but, despite persistent symmetrical synovitis, did not develop erosions. virology studies and blood and synovial fluid (SF) cultures were consistently negative; a search for crystals in the SF was unrevealing. Peripheral blood (PB) B cells were absent, whilst the PB CD3( ) cell count was normal. The ratio of naive (CD45RA( )) to memory (CD45R0( )) cells was also normal (1:1) but the CD4:CD8 ratio was reversed. To our knowledge, this is the first report which combines the immunophenotypic analysis of the PB with that of the SF and synovial membrane (SM). This confirmed the absence of B cells and the reversed CD4:CD8 ratio. However, as in other chronic arthropathies, the SF and SM cellular infiltrate consisted almost exclusively of memory T cells, consistent with the preferential localization of this subset to inflamed tissues. This case indicates that synovitis can proceed persistently in the absence of B cells and that the migratory mechanisms of T cells are not altered. However, the case suggests that the absence of B cells and negativity for rheumatoid factor, combined with an increased presence of CD8( ) (suppresser/cytotoxic) T cells in the joint, might contribute to the non-erosive nature of the synovitis.
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ranking = 0.50967651312572
keywords = membrane
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7/99. Marked gastric foveolar hyperplasia associated with active cytomegalovirus infection.

    Transient protein-losing gastropathy with marked gastric rugal hypertrophy, a Menetrier's disease-like process, is an uncommon condition of unknown etiology. Most of these cases have been reported in pediatric patients; the clinical course is usually favorable. cytomegalovirus infection has been implicated as a possible etiology in many of the reported cases. This type of Menetrier's-like disease has not been well documented in adult patients. We describe here an elderly man who presented with severe protein-losing gastropathy and large polypoid nodules in the stomach. Gastric mucosal biopsies revealed marked gastric foveolar hyperplasia with morphological evidence of cytomegalovirus (CMV) infection. Immunohistochemical analysis demonstrated an aberrant topographic distribution of staining for transforming growth factor-alpha and transforming growth factor-beta receptor II, as described in other cases of Menetrier's disease in the literature. This transient Menetrier's-like disease associated with CMV infection should be considered in the differential of both pediatric and adult patients with GI protein loss.
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ranking = 0.49032348687428
keywords = mucosa
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8/99. coronary angiography and intravascular ultrasound.

    Clinicians have long used the size of the lumen and the angiogram as a predictor of coronary events. However, cardiovascular disease is not a disease of the lumen but a disease of the vessel wall. In early stages, atherosclerosis outwardly remodels the external elastic membrane; only late in the disease process does luminal narrowing occur, enabling angiographic detection. This has profound implications for drug therapy, because approximately 70% of patients present with acute myocardial infarction (MI) or sudden death, not angina as the first symptom of coronary disease. Intravascular ultrasound (IVUS) can provide detailed images of the artery and is the only technique currently available that enables physicians to routinely visualize coronary plaques. Due to its sensitivity in measuring plaque volume and content, IVUS may be a useful surrogate marker to evaluate the atherosclerotic process in smaller numbers of patients than required for conventional clinical endpoint trials.
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ranking = 0.50967651312572
keywords = membrane
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9/99. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale.

    Eosinophilic angiocentric fibrosis (EAF) is an unusual fibrotic condition affecting the mucosa of the upper respiratory tract, of which there have been eight reported cases. The condition is thought to be associated with the rare skin disorder granuloma faciale, which is histologically identical, and was present in two cases of EAF. We report the third case where EAF and granuloma faciale occurred together, to highlight this type of intranasal fibrosis as a distinct pathological entity.
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ranking = 2.4516174343714
keywords = mucosa
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10/99. Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component.

    We report a case of chordoma containing a spindle cell sarcomatoid component with a gradual transition from conventional chordoma. Immunohistochemically, many tumor cells in both conventional chordoma and sarcomatoid components were positive for cytokeratins (AE1/AE3, CAM5.2) and epithelial membrane antigen as well as vimentin. This report provides a rare example of sarcomatoid chordoma. Familiarity with this type of bone tumor should help to avoid confusion with dedifferentiated chordoma and other spindle cell sarcomas or carcinomas.
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ranking = 0.50967651312572
keywords = membrane
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