Immunopathologic features of pemphigus in the east Mediterranean region of Turkey: a prospective study

Pemphigus, a life-threatening autoimmune disorder, is the most common autoimmune bullous disease in the Mediterranean region of Turkey. No studies have investigated the immunopathologic features of this geographic setting. To determine the immunopathologic features of pemphigus in the Eastern Mediterranean region of Turkey, the authors evaluated the histopathological, immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA) results in a 4-year study. In this prospective study, tissue from 174 patients was analyzed by direct IF (DIF); 384 by indirect IF (IIF) from 61 patients with pemphigus; and 88 by ELISA for antibodies against desmoglein (Dsg) 1 and Dsg 3 from 50 of those 61 patients. Pemphigus vulgaris (PV) was the most commonly observed subtype (46 of 61 patients, 75.41%), followed by pemphigus foliaceus (9 of 61 patients, 14.75%), pemphigus erythematosus (5 of 61 patients, 8.2%), and pemphigus herpetiformis (1 of 61 patients, 1.64%). There was a significant correlation between clinical activity score (CAS) and IgG antibody titer in IF (P < .001) and ELISA tests (P = .024 for Dsg 1; P = .028 for Dsg 3). Antibody titers and C3 scale did not predict exacerbations and relapse. The commonest clinical subtype of pemphigus was PV in this region. Results indicate that IgG antibody titer in IF and ELISA tests of patients with pemphigus are correlated with CAS; however, they are not useful in predicting exacerbations and relapse of disease.