Epidemiology of Pemphigus in Hospital Universitario Virgen Macarena, Seville, Spain, 2005-2006

Background. Pemphigus is a serious, potentially fatal chronic autoimmune bullous disease with cutaneous and mucosal manifestations. Early diagnosis and treatment are essential. Material and methods. We performed a retrospective cohort study that included patients diagnosed with pemphigus in the dermatology department of Hospital Universitario Virgen Macarena, Seville, Spain, in 2005 and 2006. We reviewed demographic, clinical, and therapeutic data. Results and conclusions. Twenty-three patients, the majority women, were included in the study. Clinical onset usually occurred between 30 and 60 years of age. The most common variant was pemphigus vulgaris (79 %), and the sites most frequently affected were the oral mucosa, trunk, and scalp. Oral corticosteroids were the initial treatment of choice in all patients, and azathioprine and mycophenolate mofetil were given as adjuvant therapy. Complete remission was induced in 8.68 % of patients and partial remission in 91.32 %. The main complications were infections, osteopenia and osteoporosis, and cataracts.