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

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.