Pemfigusta desmoglein antikor serum düzeyleri ile direkt immünofloresan bulgularının hastalığın klinik aktivitesi ile ilişkisi

Abstract

Background and Design: Pemphigus is an autoimmune disease that results in blistering of the skin and mucous membranes. In this study, we investigated the relationship between disease activity and remission with ELISA scores and direct immunofluorescence (IF) - two methods used for the detection of antibodies against desmoglein-1 (dsg-1) and desmoglein-3 (dsg-3) that are responsible for blister formation.

Material and Method: Twenty-three pemphigus vulgaris patients and two pemphigus foliaceus patients were enrolled in the study. The serum levels of anti-dsg-1 and anti-dsg-3 antibodies were measured with ELISA before therapy and at 3, 6, and 12 month of clinical remission. Concurrently, direct IF was performed on perilesional skin during active disease and on normal buttock skin/lower lip mucosa in remission. The tests were repeated if relapse has occured.

Results: Anti-dsg-1 was detected in 17 (73.9%) pemphigus vulgaris patients and anti-dsg-3 in 23 (100%) pemphigus vulgaris patients. In two pemphigus foliaceus patients, anti-dsg-1 values were positive, while anti-dsg-3 values were negative. A statistically significant correlation was seen between anti-dsg-1 antibody serum levels and skin severity scores (r: 0.577; p: 0.003), as well as between anti-dsg-3 antibody serum levels and oral mucosa severity scores (r: 0.539; p: 0.008). Direct IF results in 16 patients (84.2%) who achieved complete remission were negative. In 9 patients who relapsed, elevated serum values of anti-dsg-1 and/or anti-dsg3 were also found. Increase in serum antibody levels was detected 1-4 months before the relapse in three of them.

Conclusion: In this study, we observed that serum desmoglein antibody levels correlated with disease severity and activity. In clinical remission, serial measurements of desmoglein antibodies can provide a guide for clinical follow-up and treatment modification.