Pemphigus foliaceus, myasthenia gravis, thymoma and red cell aplasia. A case report and indirect immunofluorescence study on 38 patients with myasthenia gravis.
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Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune skin diseases caused by autoantibodies against desmoglein (Dsg) 3 and Dsg1. We have previously developed ELISAs using recombinant Dsg3 and Dsg1 expressed by baculovirus as a diagnostic tool for pemphigus. In this study, we determined the frequency of coexistence of IgA class as well as distribution of IgG subclass. Two out of 49 PV and PF sera tested had anti-Dsg1 IgA in addition to anti-Dsg1 IgG. Interestingly, one of them showed prominent pustular formation. Among IgG subclass, IgG4 was predominant and found in all of the 30 PV and 19 PF sera tested, followed by IgG1, detected in 25 out of 30 PV and 12 out of 19 PF sera. Even though IgG2 and IgG3 were detected in 13 and one PV and 6 and 4 PF sera, respectively, the ELISA titers had barely exceeded the cutoff value in most of the cases. There was no IgG subclass shift during the course of the disease in seven cases examined. These findings indicate that IgG4 subclass is the predominant autoantibodies in both PV and PF, while IgG1 is also frequently found.
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The serum hexosamine levels of 39 patients with Pênfigo Foliáceo Brasileiro were determined. The mean value obtained, compared with the mean value found in 30 normal individuals in our laboratory, by Santos et al. (1977), shows that in this disease, the levels of serum glycosamine are higher than in the normal group. The application of Student's test (/t/) indicates a significant difference in the mean values (/t/) = 9,02 P greater than 0,001). Although little is known about the fundamental processes concerned with the increase of serum glycosamines, the authors based on the suggestions of Seibert et al. (1947) reason on the possibility that the essential lesion, i.e., the destruction of the intercellular cement (of glycoprotein nature) is an important factor in the increase of glycoproteins rich in glicosamines.
BACKGROUND: Two assays are available to detect anti-skin antibodies in patients with fogo selvagem (FS): indirect immunofluorescence (IIF) and immunoblotting (IB). This study was conducted to compare the sensitivity of these assays in detecting FS antibodies. DESIGN: Eighty-nine serum samples of 48 patients with FS and control serum from 15 normal individuals were tested concurrently for the presence of FS antibodies by IIF and IB. IIF studies were conducted using four different substrates: human skin, monkey and guinea pig esophagus, and bovine tongue. RESULTS: FS antibodies were detected much more commonly by IIF than by IB, i.e. in 71% vs. 28% of serum samples respectively. By IIF, the antibodies reacted most strongly against human skin. CONCLUSIONS: IIF is a more sensitive assay than IB for detecting antibodies associated with FS. The sensitivity of the test is maximized by using human skin as a substrate.
BACKGROUND: Fogo selvagem (FS) is an autoimmune disease that is endemic in certain regions of Brazil and appears to be precipitated by an environmental factor. OBJECTIVE: Our purpose was to confirm the occurrence and prevalence of FS in a population of Xavante Indians living in an endemic region of central Brazil. METHODS: Clinical, anthropologic, and immunologic studies were carried out in patients and in normal inhabitants of the Pimentel Barbosa Indian Reservation, Mato Grosso, Brazil. RESULTS: FS was identified and confirmed in 10 patients from a patient pool of 295 with various skin diseases. The Xavante settlement has a total population of 746. Anti-desmoglein 1 autoantibodies were detected in all patients with FS and were absent from more than 300 serum samples collected from randomly selected unaffected persons. CONCLUSION: FS is strongly linked to outdoor activities and is largely restricted to immunogenetically predisposed persons. FS appears to have been endemic in certain regions of South America for several centuries.
BACKGROUND: Fogo selvagem (FS) is an autoimmune intraepidermal blistering disease mediated by antidesmosomal autoantibodies. Patients with FS do not have mucosal lesions despite extensive skin involvement. OBJECTIVE: Our purpose was to evaluate the epidermis and the oral epithelium of patients with FS as targets of antidesmosomal autoantibodies. METHODS: Fifteen patients were studied clinically, histologically, and immunologically. Biopsy specimens from the skin and the oral mucosa were studied by light microscopy and direct immunofluorescence. The serum of each of these patients was also titrated by indirect immunofluorescence. RESULTS: All patients showed skin lesions and subcorneal acantholyis, but none exhibited oral blisters or erosions. Direct immunofluorescence analysis demonstrated the presence of tissue-bound autoantibodies in both the epidermis and the oral epithelium of all patients with FS. Antiepidermal autoantibodies were also found in the sera of the patients. CONCLUSION: Relevant epitopes on desmoglein 1 molecules of oral epithelium may not be available to react with pathogenic FS autoantibodies.