Background: Autoimmune bullous diseases are organ-specific diseases characterized by autoreactive T and B cells specific for structural proteins of the skin. The incidence and prevalence of autoimmune blistering diseases vary in different countries and their epidemiology has not yet been addressed in Romania. Methods: In this study between 2001 and 2007, we prospectively investigated a total of 116 patients with autoimmune blistering diseases from the Northwestern region of Romania. The diagnosis was based on the clinical, histo- and immunohistological as well as serological findings. Results: Pemphigus was the most common disease representing 58.6% of the case (68 cases); 40 cases (34.5%) were diagnosed with bullous pemphigoid (BP) and eight cases (6.9%) with other autoimmune sub-epidermal diseases. The incidence and prevalence of pemphigus diseases were four patients/1 000 000 inhabitants/year and 0.00248%, respectively. BP occurred in 2.5/1 000 000 inhabitants/year and its prevalence was 0.00146%. While the average onset age for pemphigus vulgaris was 53 years, BP patients were first diagnosed at a mean age of 73.6 years. Conclusion: The genetic background of the local population may explain why pemphigus occurs more commonly than BP in Northwestern Romania compared with the population of Western Europe. In addition, the shorter life expectancy in Romania (71.3 years) compared with Western Europe (>80 years) may contribute to this phenomenon.