Epidemiology of pemphigus in the western region of Turkey: retrospective analysis of 87 patients

Abstract

Background: Pemphigus is a worldwide disease and varies in its clinical profile and epidemiology in different regions of the world. The disease is rare and few epidemiological data are available.Objective: The purpose of this study was to evaluate the epidemiologic, clinical, and therapeutic features of pemphigus in the western region of Turkey.Methods: We retrospectively investigated a total of 87 patients with pemphigus. The parameters including age at diagnosis, sex, diagnostic delay, diagnostic methods, hospitalizations, distribution of skin lesions, duration and dose of therapy, side effects, and duration of follow-up were evaluated.Results: The mean age at the onset was 48 years. The average annual incidence of the disease was seven new patients and estimated annual incidence rate was 0.18 per 100,000 inhabitants. The male-to-female ratio was 1/1.64. All patients were treated with systemic corticosteroids. The mean initial dose was 100 mg prednisolone equivalent. Thirty-three (37.9%) patients received azathioprine as adjuvant therapy. The mean time to control the disease activity was 23 days in our study group. Ten patients (11.5%) were in clinical remission without treatment, 41 patients (47.1%) were on low dose steroid (≤10 mg daily), and 28 (32.2%) were using >10 mg/day corticosteroid at the end of the study period.Conclusion: Pemphigus vulgaris (PV) is the most common clinical subtype in the western part of Turkey. The disease is more frequent in the fifth decade of life and has a female predominance in our region. The patients with both oral mucosa and skin involvement need higher corticosteroid dose, more time to control the disease activity and longer hospitalization time when compared with the patients with oral mucosa involvement alone.