Factors causing clinical and / or serological activation inpatients with pemphigus foliaceus and its effects ontreatment process

Objective: Pemphigus foliaceus is a rare and chronic autoimmune bullous disease characterized by superficial bullae. It is believed that the onset and course of the disease is affected by environmental and personal factors. Although large studies on the pemphigus group of the diseases in heteregenous groups for long follow-up periods have been available in the literature, pemphigus foliaceus which is relatively rare, just includes a small portion of these study groups. We aim to determine the epidemiological characteristics of the patients in a large series of cases and to investigate the factors affecting the onset and course of the disease, their effects on the course of the treatment and to contribute to the literature. Methods: We retrospectively reviewed the files of patients who were diagnosed clinically, histopathologically and / or serologically with pemphigus foliaceus in our outpatient clinic of bullous diseases of Istanbul University Medical Faculty, Department of Dermatology between 1980 and 2017. We analyzed their gender, age at the time of the diagnosis, habits, locations of residency and homeland, other demographical characteristics, clinical characteristics such as the duration of the complaints, distribution of the lesions, treatments given, clinical and serological parameters during the course of the disease, activation periods if exist, reasons responsible for activations, and effects of environmental and personal factors. All findings were recorded in the SPSS program and statistically analyzed using appropriate methods. The results were discussed in comparison with other studies reported in the literature. Results: A total of 65 patients consists of 32 women (49.2%) and 33 men (50.7%), who were diagnosed with pemphigus foliaceus, were identified during the years studied. The mean age of the patients was 45.63±14.78 years at the time of diagnosis. 26% of patients had smoking and 6% had habit of alcohol abusing. When the patients were evaluated according to their geographical regions of the residency and homelands, the Marmara region ranked first. Twenty patients had additional disease (diabetes, hypertension, hypothyroidism, depression and other) during the diagnosis. The mean duration of the current complaints at the diagnosis was 1,72±2,81 months. In spring and summer, the total amount of the onset of the disease was higher than that seen in autumn and winter (p=0.09). After the diagnosis, 11 patients were lost to follow-up and the mean follow-up period was calculated as 5,61±1,95 years in the rest of 4 the group. In all patients, systemic corticosteroids were used as the first treatment and in 35 patients an additional treatment (azothiopyrine, mycophenolic acid, dapsone, rituximab, intravenous immunoglobulin and photopheresis) was started. 24 patients had at least one complication due to treatment. A total of 109 activations in thirty-nine patients were most frequently observed in the summer season (n=31). In 43 activations, the causes were upper respiratory tract infections (n =16), misuse of the drugs (n=11), stress (n=9), fasting (n=3) and other infections (n=4). For the 66 activation that could not be associated with a specific reason, the total number of activation (n=43) in the spring + summer seasons was statistically significantly higher than the number of activation (n=27) in autumn + winter (p=0.027). There were no significant differences in terms of gender, age of diagnosis, smoking habits, alcohol abuse and time to diagnosis between patients with at least one activation and no activation. The highest values measured for each of the 22 patients who were monitored with anti-Desmoglein 1 measurements at the intervals were highest in spring and summer seasons when evaluated according to seasons (p>0.05). Although the average of anti-Desmoglein measurements were higher in spring + summer seasons compared to other seasons, the difference was not significant. Discussion: Our study was the first demographic study in a large group of isolated and non-endemic pemphigus foliaceus patients, and also, the first study to investigate the effect of environmental and personal factors on the onset and course of disease. The proportion of patients with pemphigus vulgaris between the years they were examined and their demographic characteristics such as age and gender are consistent with the literature. In our study, a significant relationship was found only between the seasonal changes and the activations among many parameters. In the previous limited number of studies examining patients with heterogeneous pemphigus group, there are different results in terms of seasons when the onset of the disease is most common. In the literature, there were no significant results in the only study which also investigated activation-season relationship. In our study, we found both activations and the onset of the disease more frequently in spring and summer seasons, but only the activation-season relationship was statistically significant. When the anti-Desmoglein 1 values measured by ELISA were used in the follow-up, there were remarkable results showing that they may be affected by the seasons. Keywords: Pemphigus foliaceus; pemphigus erythematosus; bullous diseases; enviromental factors; activation