Skin hyper-reactivity in patients with benign intracranial hypertension as an early manifestation of Behçet's disease.
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Biomedical subjects
Publications and source records attributed to J A Bosch.
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Labial salivary gland tissues from 20 patients with primary Sjögren's syndrome (group 1), 26 with the secondary type of the disease (group 2), 16 patients with the incomplete form of the disease and 10 normal controls were examined for the presence of lymphocyte infiltrates, epithelial damages and plasma cell aggregates. Activated T lymphocytes of the helper phenotype predominated in groups 1 and 2. Aberrant epithelial expression of HLA class II molecules was found in the same group of patients, while IgG- and IgM- containing plasma cells were detected.
BACKGROUND: The idiopathic hypereosinophilic syndrome (IHS) is a rare entity of unknown etiology. Hematological and cardiac involvement is predominant. A series of 12 patients with this syndrome, initiated in 1982, is described. METHODS: Cardiological study by repeated echocardiograms and hematological study in peripheral blood and bone marrow upon initiation of the disease were performed. RESULTS: Median follow up was of 48 +/- 31 months. Males predominated (75%) with mean age being 55 +/- 15 years. The principal organs or systems involved were the heart (50%) and the nervous system (41%). Of the 6 cases with cardiac involvement only 2 had clinical manifestations. The remaining 4 patients were diagnosed from echocardiographic changes with the principal alterations observed being: atypical occupation of the ventricles, endocardial thickening and mitral and tricuspid subvalvular cumulus. Echocardiographic follow-up only showed changes in one case. Hematological involvement was characterized by moderate leukocytosis with hypereosinophilia formed by mature eosinophils, conservation of other hematopoietic series, absence of blasts in peripheral blood, finding suggestive of diseosinophilopoiesis and appearance of myelofibrosis and cytogenetic alterations. Survival at four years was 58%. CONCLUSIONS: In the series studied cardiac involvement is frequent, being principally diagnosed in a subclinical phase and with a very slow echocardiographic evolution. At a hematological level changes typical in myelodysplastic syndromes and myeloid leukemia were observed.
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A series of 31 patients suffering idiopathic inflammatory myopathy (IIM); we describe the extramuscular manifestations, specially pulmonary, the association to neoplasia, the histopathological characteristics, and their response to treatment. Fourty three percent of IIM patients presented a pulmonary involvement, 9% presented an associated neoplasia. The histopathological study allowed us to clearly differentiate dermatomyositis and polymyositis within IIM. 65% of patients initially responded to glucocorticoids and the most usefull therapeutic alternatives were azatioprine and cyclosporin-A.
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Two patients with Hodgkin's disease developed a condition that has been termed "reactive perforating collagenosis." In both cases, pruritus was severe and seemed to be responsible for the vigorous rubbing and scratching that caused the skin lesions. In one patient, the lesions disappeared upon remission of Hodgkin's disease. Perforating collagenosis has been described in children as a hereditary disease, and has also been described in adults with diabetes and on hemodialysis. On the basis of the changes seen in our two patients with Hodgkin's disease, we suggest that perforating collagenosis is simply a consequence of intense scratching.
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In order to know the usefulness of beta-2-microglubulin as a tumor marker, the authors measured by radioimmunoassay its serum concentration in 222 patients with solid tumors of different localization. 116 patients with hematologic neoplasms, and 254 cases grouping healthy individuals and patients with diseases other than cancer. The upper limit of normality was considered as 3 micrograms/ml; 35.6 per 100 of the investigated solid tumors and 43.8 per 100 of the blood-forming organs neoplasms gave values higher than 3 micrograms/ml, against only 5 and 10 per 100 of the healthy and non-cancer patients respectively used as a control. Many cancer patients did not show an augmentation of beta-2-microglobulin. The authors consider that beta-2-microglobulin does not offer enough sensitivity and specificity to be used as a tumor marker.
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