Nineteen-year retrospective evaluation of pemphigus in a single dermatology centre in Istanbul, Turkey

Abstract

Introduction: Pemphigus is an autoimmune intra-epidermal bullous disease of the skin and mucosae. Aim: To retrospectively evaluate the course, prognosis and clinical features of pemphigus. Material and methods: The files of 196 pemphigus patients admitted to our clinic between December 1995 and December 2014 were collected and analysed. Results: The male to female ratio among patients was 1 : 1.88. Pemphigus vulgaris (PV) was the most common clinical variant observed in 175 (89.3%) of the patients, followed by pemphigus foliaceus (PF) in 14 (7.1%) of the patients. The mean patient age at disease onset was 50 years. PV presented itself as skin lesions in 55 (31.4%) of the patients and as oral mucosa lesions in 120 (68.6%) of the patients. Complete remission and treatment withdrawal were obtained in 112 (57.1%) of the patients, for a mean period of 2.91 +/- 2.66 years (range: 4 months to 13 years). The mortality rate was 6%, and relapse occurred in 16 (14.3%) of the patients for a mean relapse period of 2.15 +/- 1.88 years (range: 6 months to 7 years). Mucocutaneous pemphigus (MCP) was the major clinical pattern observed in 96 (49%) of the patients. Conclusions: Within our study population, pemphigus predominately affected females, and the most common clinical variant was PV, a subtype that frequently occurs in middle-aged individuals. MCP was the most common clinical pattern. Although MCP and higher doses of corticosteroids were needed to control pemphigus, they did not seem to influence the prognosis.