ODSS vs. ABSIS and PDAI oral parts in pemphigus vulgaris: inter-rater reliability and testing times.

OBJECTIVES: The existence of standard methods for diagnosis and measuring the severity of diseases leads to a more accurate severity assessment, the possibility of following up, and the possibility of comparing the results of studies. This study aimed to compare different pemphigus vulgaris (PV) assessment methods regarding inter-observer reliability and testing times-focusing on oral parts. MATERIALS AND METHODS: Two dermatologists evaluated orally involved PV patients by oral parts of Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Oral Disease Severity Score (ODSS). RESULTS: Seventy patients completed the study. The intraclass correlation coefficient showed the evaluators' agreements on ABSIS, PDAI, and ODSS with 0.98, 0.94, and 0.95, respectively. Reliability analyses showed near-perfect relationships between each scoring methods pairs. There was no association between lesion sites and disease severity. The PDAI scoring duration was significantly shorter, and the ABSIS scoring duration was significantly longer. CONCLUSION: ODSS is valid for evaluating oral involvement in patients with PV and relates to ABSIS and PDAI almost perfectly. Besides, it was shown that the evaluation of patients' oral involvement based on PDAI and ODSS is done in about 1 min, which seems clinically reasonable.

Indirect immunofluorescence on rat bladder epithelium in patients with pemphigus vulgaris with an extended follow-up.

Background: Indirect immunofluorescence (IIF) on rat bladder epithelium (RBE) has been widely used to detect anti-plakin antibodies present in paraneoplastic pemphigus (PNP). However, anti-plakin antibodies have also been found in a group of patients with pemphigus vulgaris (PV). Objectives: To assess the reactivity rate of PV sera in IIF using RBE as substrate and the diagnostic usefulness of the aforementioned test. Methods: Patients diagnosed with PV presenting to Razi Hospital, Tehran, Iran, were recruited. The patients' demographics, disease severity, and response to the initial treatment were recorded. Sera were collected and tested by IIF on RBE and by desmoglein 3/1 (Dsg 3/1) enzyme-linked immunosorbent assay. Patients were followed up closely for a mean of 53.9 months for any evidence of malignancy. Results: Forty-six patients were enroled (mean age of 42.9 years old, 31 females). Nine sera (19.6%) showed reactivity in IIF on RBE. Mean serum anti-Dsg levels did not differ significantly among the two groups with positive and negative IIF results. Negative anti-Dsg3 was related to a higher positive rate in IIF on RBE. There was no significant correlation between the reactivity of IIF on RBE and patients' demographic, clinical, or serological characteristics. Conclusions: IIF on RBE is a sensitive test for detecting antibodies against plakins. However, it has a relatively high false-positive rate in PV, probably due to the epitope spreading phenomenon. This test should be suggested when there is a clinical or immunohistopathological suspicion of PNP and should be interpreted with caution.