Epidemiology of autoimmune bullous diseases and therapeutic modalities during a 10 year period in Iran.

Autoimmune bullous diseases are systemic disorders with autoantibodies that result in blisters. Aim of this study was to indicate the spectrum and treatment modalities of five types of bullous disorders most prevalent in the south of Iran: pemphigus vulgaris (PV), pemphigus foliaceus (PF), epidermolysis bullousa aquisita (EBA), bullous pemphigoid (BP), and pemphigoid gestationis (PG). Patients with PV, PF, BP, EBA, and PG were included in this study. The data regarding the age, sex, and the treatment used for PV, PF, and BP were recorded and analyzed in our center, a tertiary referral center. T-test and Mann-Whitney test for independent samples were used for the analysis of parametric and nonparametric variables, respectively. Chi-square test was used for frequencies. Of the 441 patients included in this study, 82.9% had PV, 4.7% PF, 8.5% BP, 1.5% EBA, and 1.3% PG. 93.5% of patients with PV, 95.3% with PF, and 100% with patients with BP were treated and responded to first line therapies with one or two medications. The most frequent autoimmune bullous disease was PV, followed by PF. For PV and PF, combination of prednisolone and azathioprine was the most frequent first line medication. In the patients with BP, prednisolone monotherapy was the most frequent one. Only a minority of patients with PV and PF needed the third or fourth medications.