Cli̇ni̇cal and demographi̇c features of pati̇ents wi̇th pemphi̇gus at Di̇cle Uni̇versi̇ty Faculty of Medi̇ci̇ne i̇n 2005-2012

Pemphigus is a chronic, autoimmune, blistering disease of the skin and mucous membranes. The disease shows variable incidence, clinical and epidemiologic characteristics in different parts of the world. Therefore, the aim of this study was to examine the clinical and epidemiologic features of pemphigus in our geographic region. A total of 122 cases of pemphigus diagnosed in the Dermatology Clinic at the University of Dicle during the period between 1 January 2005 and 31 December 2012 were studied prospectively and/or retrospectively. Of the 122 patients, 54 were males and 68 were females. The male to female ratio was 1:1.25. There was a distinct female predominance among the patients with pemphigus vulgaris with a male to female ratio of 1:1.45. The mean age at the onset of the disease was 45 years for all patients with pemphigus (range 9-81). Within the Diyarbakır Province, 53 new cases of pemphigus determined during at the study period, and estimated annual incidence rate was 4.4 per 1 million inhabitans. The major clinical form of the disease was pemphigus vulgaris, which was observed in 103 patients (%84.4) followed by pemphigus foliaceus observed in 12 patients (%9.8). In the pemphigus vulgaris group, the site most frequently involved was the oral mucosa in 95 patients (%92.2). The mean lag time between the onset and final diagnosis was 126.7 days (range, 1 week to 4 years). Among pemphigus vulgaris patients, the mean lag time was 127.2 days. The most prevalent previous or concurrent diseases were osteoporosis (17 case), diabetes mellitus (13 case), psychiatric disorders (10 case) and ischemic heart diseases (10 case). The initial treatment in 120 of patients was systemic corticosteroids at an average dose of 1 mg/kg/d. The most common adjuvant immunosuppressants used were azathioprine (106 patients, %86.8), followed by dapsone (10 patients, %8.1) and mycophenolate mofetil (8 patients, %6.5). Osteoporosis and cushingoid changes were the most frequent side-effects observed in 30.3% and 18.8% of patients, respectively. Urinary tract infections (%13.9) and hyperglycemia (%6.5) were other notable side-effects of treatment. During the study period of 8 years 8 patients (%6.5) died, four of whom (%3.8) were considered to be treatment related deaths. All of the cases who died were pemphigus vulgaris patients, the most frequent cause of death was sepsis. KEYWORDS, Pemphigus, epidemiology of pemphigus, management of pemphigus, cilinical features of pemphigus