Autoimmune bullous diseases seen in patients who applied to Dicle University Medical Faculty Hospital Dermatology Polyclinic

Introduction and Aim: Autoimmune bullous diseases (AIBD) are a rare group of diseases characterized clinically by vesiculobullous lesions on the mucous membranes, primarily the oral mucosa, and/or skin, as a result of the formation of autoantibodies against various autoantigens in the skin and/or mucous membranes. Our aim was to determine the number and rate of all the patients diagnosed an autoimmune bullous disease for the first time who applied to the dermatology outpatient department and/or were hospitalized in the dermatology clinic during a one-year period. Since Dicle University Medical Faculty Hospital is regarded as one of the reference centers in the Southeastern Anatolia Region, it was aimed to obtain information about the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. Materials and Methods: In this study, a total of 52 patients who applied to the Dicle University Faculty of Medicine Dermatology and Venereal Diseases polyclinic and/or were hospitalized in the dermatology clinic between May 4, 2020 and May 4, 2021 were evaluated. The patients included in the study were the ones diagnosed with an autoimmune bullous disease for the first time without age restriction and any exclusion criteria, and signed an informed consent form before inclusion in the study. Results: A total of 52 patients diagnosed with autoimmune bullous disease for the first time during the specified period were evaluated in this study. It was determined that 27 (51.9%) of the patients were female, 25 (48.1%) were male, and their mean age was 52.67±25.262 (years). Twenty-eight (53.8%) of the cases were in the pemphigus group and 24 (46.2%) were in the pemphigoid group. When examined according to autoimmune bullous diseases subgroups, it was seen that in the pemphigus group; 22 patients (42.3%) had pemphigus vulgaris, 5 patients (9.6%) had pemphigus foliaceus, 1 patient (1.9%) had paraneoplastic pemphigus, and in the pemphigoid group; 19 patients (36.5%) had bullous pemphigoid, 3 patients (5.8%) had linear IgA dermatosis, 1 patient (1.9%) had acquired epidermolysis bullosa and 1 patient (1.9%) had chronic bullous dermatosis of childhood. In our study, the most frequently seen autoimmune bullous disease (AIBD) in the patients admitted to our clinic was pemphigus vulgaris with a rate of 42.3%, and bullous pemphigoid with a rate of 36.5% was the second most common. The ratio of all pemphigus cases to bullous pemphigoid cases was 1.47. Nine of the pemphigus vulgaris patients and 12 of the bullous pemphigoid patients were residing in Diyarbakır. It was determined that the incidence of pemphigus vulgaris in Diyarbakır was at least 5.00 per million, and the incidence of bullous pemphigoid was at least 6.66 per million. The mean age of bullous pemphigoid patients was 72.84±10,495 (years), and the mean age of pemphigus vulgaris patients was 42.55±19,680 (years). When the cases were examined in terms of other diseases accompanying autoimmune bullous disease; hypertension in 23.1%, endocrinopathy in 21.2%, cardiological disease in 9.6%, neurological disease in 5.8%, asthma/COPD in 5.8%, other skin diseases in 3.8%, malignancy in 1.9%, and hematological diseases in 1.9% were determined. A statistically significant association was found between bullous pemphigoid and hypertension, endocrinopathies and cardiological diseases (p<0.05). A statistically significant association was also found between pemphigus vulgaris and hypertension, endocrinopathies and cardiological diseases (p<0.05). Conclusion: There are rare prospective epidemiological studies in the literature comparing pemphigus and pemphigoid group diseases with their comorbid conditions. This study we conducted contributed to the annual incidence and epidemiology of autoimmune bullous diseases in Diyarbakır and surrounding provinces. The fact that bullous pemphigoid develops in the elderly population and medical comorbidities have a higher prevalence in this population may explain why these diseases occur together; however, whether some common pathways are involved in their pathogenesis is open to research. Further studies are needed to understand whether certain diseases are more common in patients with pemphigoid and pemphigus due to common pathophysiological pathways or whether they are purely coincidental. Keywords: Autoimmune bullous disease, pemphigus, pemphigoid, epidemiology