Background: The purpose of this study was to describe the epidemiological and clinical features, course, response to treatment, and prognosis of pemphigus in the Mediterranean region of Turkey.
Methods: All patients with confirmed pemphigus were prospectively enrolled in two major dermatology departments in the cities of Adana and Antalya in the Mediterranean region between March 1998 and March 2004. Details including demography, findings of clinical examinations, treatment, course, and prognosis were recorded.
Results: One hundred and forty-eight patients with pemphigus were diagnosed during the 6-year period, with a prevalence of 1.46 and an annual incidence of 0.24 per 100,000 in this region. There was a female predominance with a male to female ratio of 1:1.4. Pemphigus vulgaris (PV) was the most common clinical subtype, identified in 123 patients (83%). The mean age of onset was 43. In 101 (82%) patients with PV, disease began as persistent oral ulcers. The majority of the patients with PV could be managed with middle or high-dose steroids (60-140 mg/day). Complete clinical remission was obtained in 41 (39.4%) patients. The mortality rate was 4.8%.
Conclusions: A moderately high incidence of pemphigus was found in the Mediterranean region of Turkey as compared with that encountered in other countries. The commonest clinical subtype was PV with a 9.5-fold higher incidence than pemphigus foliaceus. It is more frequent in middle-aged people and has a female predominance. Although a relatively higher dose of steroid was needed to control the PV, the disease completely remitted in a significant proportion of the patients.