[Blood coagulation system in patients with certain dermatoses].
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Human peripheral blood lymphocytes (PBL) are activated to become suppressor T cells (S-T-C) by incubation with Concanavalin-A (Con-A). This has become the standard method for evaluation of suppressor function in patients. S-T-C function has been found to be impaired in several autoimmune diseases, including systemic lupus erythematosus (SLE). Using this assay, we have investigated suppressor-cell function in 2 autoimmune disorders, bullous pemphigoid (BP) and pemphigus vulgaris (PV), studying 6 patients from each group. Three patients with active SLE (positive controls), and 11 normal donors (negative controls) were also included. None of these patients had received systemic therapy with the exception of 2 patients with PV who were treated with gold in the past. PBL from these patients were incubated with and without 40 micrograms/ml Con-A for 72 hr to generate suppressor cells. Both groups of PBL were then irradiated wih 1500 r cobalt. Co-cultures were set up in sextuplicate using normal PBL as responders. Responder PBL were stimulated with 0.5, 1.0, and 2.0 micrograms/ml of phytohemagglutin (PHA) and 5.0, 10.0, and 20.0 micrograms/ml of Con-A. Cultures were pulsed on day 3 with 3H-thymidine and harvested on day 4. Data were analyzed using Student's t-test. S-T-C function was found to be significantly impaired in SLE vs normal (p = 0.0316). No statistically significant difference was seen in BP (p = 0.5883) and PV (p = 0.0921) as compared with normals. A defect in suppressor cell function may still be present in patients with PV and BP for the defect may be antigen-specific and therefore remain undetected by the Con-A suppressor assay.
BACKGROUND: Pemphigus is an autoimmune bullous disease characterized by the presence of antidesmoglein autoantibodies. However, the mechanism of its autoantibody production remains unknown. In previous reports, we have described rare cases of pemphigus and pemphigoid associated with silicosis. It is well known that during long-term silicosis, some autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus or rheumatoid arthritis, can occur. OBJECTIVE: The aim of this study was to explore the presence of pemphigus or pemphigoid autoantibodies in silicosis patients without clinical bullous diseases or collagen diseases. METHOD: The presence of pemphigus antibodies was examined in 54 silicosis patients with no associated bullous diseases, using immunofluorescence, the enzyme-linked immunosorbent assay (ELISA) for desmoglein 1 and 3, and immunoblotting methods. In the antibody-positive cases, HLA genotyping of peripheral lymphocytes was performed with PCR-RFLP. RESULTS: Seven out of the 54 patients were found to be positive for pemphigus antibodies and 1 for bullous pemphigoid by immunofluorescence. In addition, by ELISA, 6 patients were found to be positive against the desmoglein 1 antigen, 2 against the desmoglein 3 antigen and 2 against both desmoglein 1 and desmoglein 3. CONCLUSION: The results of the present study strongly suggest the occurrence of pemphigus and pemphigoid autoantibodies in patients with silicosis. It remains unclear whether such patients will develop an autoimmune bullous disease in the future. Accordingly, long-term follow-up of antibody-positive patients is required.
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During a survey period of 28 years a total of 449 patients suffering respectively from pemphigus, systemic lupus erythematous and chronic nephritis was admitted to the hospital. Of the 286 patients who received glucocorticoid treatment 28 were found to have steroid diabetes (9.8%). The incidences of steroid diabetes in these diseases were as follows: pemphigus 20% (7/35), systemic lupus erythematous 12.5% (14/112), chronic nephritis 5% (7/139). Two thirds of the diabetic subjects appeared asymptomatic, while the remainder showed polydipsia and polyuria. Renal glucosuria was seen in 3 cases and hyperosmolar hyperglycemia in 4. Blood glucose level in 10 out of the 21 remaining cases was 8.9 +/- 1.5 mmol/L and in the other 11 cases 14.8 +/- 2.4 mmol/L. Generally, the treatment of steroid diabetes is not intricate. Satisfactory improvement was seen in about 80% of the patients if a strict line of therapy against primary diabetes was oriented. In this series 18 patients did well under the treatment either with reduction of steroid or with use of oral hypoglycemic agents or insulin or both. Their blood glucose levels returned to normal and urine sugar disappeared rapidly. Four of the 5 deaths were caused by their primary diseases. One died of pemphigus complicated with infection and shock exhibiting an ante mortem blood glucose level as high as 31.7 mmol/L, obviously hyperosmolar status being the predisposing factor. Due to the fact that most of the steroid diabetic patients were clinically asymptomatic, delayed or misbranded diagnosis was not infrequently seen.(ABSTRACT TRUNCATED AT 250 WORDS)
A patient with pemphigus vulgaris is presented. In addition to the intercellular deposits of immunoglobulin and complement in the Malpighian layer which is consistent with pemphigus vulgaris, concomitant granular deposits of IgG and C3 were demonstrated at the dermal epidermal junction, similar to the findings in lupus erythematosus.
Sera and frozen biopsy specimens taken for immunofluorescence can be of diagnostic value in studies of vesiculobullous eruptions and connective tissue diseases. Appropriate handling of specimens is important. In cases of pemphigus and pemphigoid, findings of typical antibodies in sera and immunoglobulin deposits in skin sections serve to establish the diagnoses. Fluctuations in titers of pemphigus antibodies afford a prognistic guide. IgA deposits at the dermoepidermal junction in patients with vesiculobullous eruptions are diagnostic of dermatitis herpetiformis. Elevated titers of antinuclear antibodies, frequently with a peripheral pattern, and DNA antibodies as well as junctional deposits of immunoglobulins or complement or both in the apparently uninvolved skin are highly characteristic of systemic lupus erythematosus, more or less typical patterns of immunoglobulin and complement deposits in the lesion appear in discoid lupus erythematosus, porphyrias, and some other dermatoses.
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To determine whether reproducible results from direct immunofluorescent tissue staining could be obtained after storing the slides at room temperature. We examined the original slides of 22 cases noted to be positive for direct immunofluorescence. Diagnoses include pemphigus, pemphigoid, lupus, dermatitis herpetiformis, lichen planus, and vasculitis. These specimens, initially evaluated during the period January 1997 to September 1998, were prepared with a standard immunofluorescence staining technique, and then a permanent aqueous mounting medium was added. All specimens were stored at room temperature in vertical slide holding trays. We focused on the presence and relative intensity of the immunofluorescence staining, as well as the final diagnosis. We then compared our readings to that of the original reports. Twenty of the 22 cases studied (91%) were read as having the same diagnosis as the initial immunopathology report. Seventeen of the 22 cases (77%) were found to have the identical or slightly less fluorescence intensity. The original reports in 3 of the cases did not comment on the original intensity of fluorescence. Thus, a comparison of fluorescence preservation could not be made. In 2 of the cases, the quality of tissue preservation was poor, and though fluorescent staining was noted, we were unable to render a diagnosis. Our results suggest that direct immunofluorescent studies, using a permanent aqueous mounting medium, can be stored over long periods of time at room temperature without significant degradation of staining.
We retrospectively analyzed direct immunofluorescence (DIF) findings from 130 cases of oral mucosal disease. The diagnosis of each case was based on history, clinical features, histopathology, and clinical follow-up. To avoid circular reasoning, we did not use the DIF results in forming the diagnoses. Our results indicate that the presence of characteristic fluorescent patterns produced by several DIF reagents can establish the diagnosis of the oral lesions of pemphigus and pemphigoid and strongly indicate the diagnoses of lichen planus and lupus erythematosus. The absence of these fluorescent patterns can help to rule out these conditions, thereby strengthening the diagnoses of other oral mucosal diseases. The results of DIF are sufficiently distinguishing to be routinely helpful as diagnostic criteria for chronic ulcerative diseases of the oral mucosa.
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Chronic ulcerative diseases of the oral mucosa are commonly seen in clinical practice. On clinical and histological appearance, the lesions may be hard to differentiate from each other. The establishment of definite diagnosis is essential because the patient may require different management and have widely varying prognosis. Immunofluorescence studies aid greatly in the process of determining the diagnosis of a number of chronic ulcerative diseases. This article reviews these chronic ulcerative diseases, describing their clinical, microscopic, and immunofluorescence characteristics. Methods of diagnosis and management of the diseases also are discussed.
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The principle of immunoelectronmicroscopic studies using horseradish perpoxidase is described. This method, especially the peroxidase-antiperoxidase multistep technique, reveals more details about the exact localization of immunophenomena in different dermatological diseases. The results of immunological investigations performed on the ultra-structural level in bullous diseases, lupus erythermatosus, vasculitis, and psoriasis are summarized and compared with the immunofluorescent and classical electromicroscopic findings.