The patient behaved clinically as a typical case of PG exhibiting a good response to moderate dose of oral corticosteroids, and lacked conventional clinical manifestation of pemphigus (30). It was evidenced that IgG4 is the major subclass of autoantibodies in active pemphigus (31C33). of 5.3 years contributing 280.8 person-years. Thirty patients (81.1%) were eventually diagnosed clinically and immunopathologically with subepidermal autoimmune bullous diseases, whereas the remaining patients (18.9%) were diagnosed clinically and histologically with other inflammatory dermatoses, but none of them developed pemphigus during the follow-up duration. Of note, 7.0% (= 23) of all patients diagnosed with bullous pemphigoid (BP) in the same period (= 328) were tested positive for IgG intercellular antibodies. Histopathological review of the biopsy specimens of these patients did not reveal acantholysis. In conclusion, the predictive value of negative test in IIF on monkey esophagus is particularly reliable to exclude a diagnosis of pemphigus. Individuals tested positive for intercellular antibodies without an initial overt pemphigus did not show an increased risk for developing pemphigus subsequently. A sizable fraction of patients with BP showed circulating intercellular autoantibodies by IIF, without Rabbit polyclonal to LIPH a histopathological evidence for acantholysis. = 106; 60.2%), 1-Methyladenine followed by isolated mucosal disease (= 63; 35.8%) and isolated cutaneous disease (= 7; 4.0%). The demographic and clinical characteristics of the patients with pemphigus included in the analysis are exhibited in Table ?Table11. Table 1 Demographic and clinical characteristics of patients with pemphigus whose sera were tested by indirect immunofluorescence microscopy. = 174)= 29)= 0.925). Patients with isolated cutaneous disease had lower sensitivity (57.1%; 95% CI, 18.4C90.1%), but the small size of this 1-Methyladenine subgroup (= 7) hinders drawing meaningful comparisons. The sensitivity of IIF on monkey esophagus in PF patients was only 69.0% (95% CI, 49.2C84.7%; Table ?Table2).2). Taken together, the sensitivity of this immunoassay was significantly higher for the diagnosis of PV as compared to that of PF (= 0.018). Table 2 Evaluation of indirect immunofluorescence immunoassay on monkey esophagus. = 23) of all patients diagnosed with (BP) in the same period (= 328) were tested positive for IgG intercellular antibodies by IIF on monkey esophagus (Physique ?(Figure11). Open in a separate window Physique 1 Flowchart of the clinical characteristics of non-pemphigus patients tested positive for intercellular cell surface antibodies in IIF on monkey esophagus. The remaining 7 patients were diagnosed clinically and histopathologically with various inflammatory dermatoses; 4 patients with atopic dermatitis, 2 patients with erythema multiforme major, and one patient with pyoderma gangrenosum (Physique ?(Figure11). None of the above 37 seropositive patients have subsequently developed pemphigus throughout the duration of follow up. Lesional biopsy specimens of 32 patients with the false-positive results were reviewed, and no histopathological sign of acantholysis was detected. Discussion The results of the current study indicate that IIF microscopy on monkey esophagus is usually a more sensitive test for the detection of circulating intercellular antibodies in PV than in PF. The specificity of the assay is usually high when used in routine practice, and the predictive value of a negative test is particularly reliable to exclude the diagnosis of pemphigus. Individuals tested positive for intercellular antibodies by IIF, without initial overt pemphigus, did not show an increased risk for developing pemphigus subsequently. None of the false-positive patients had histological evidence of acantholysis. The positivity rate of IIF in previous cohorts varied depending on the epithelial substrate 1-Methyladenine which the sera were incubated with. In a recent German study, intercellular epithelial staining on monkey esophagus by IIF was observed in 100.0 and 98.0% of 65 patients with PV and 50 patients with PF, respectively (5). In.
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