Pemphigus vulgaris (PV) is a bullous disease characterized by the presence of autoantibodies against intra-epidermal adhesion molecules. AIM: The aim of this study was to identify the characteristics of PV in each gender, forms of clinical presentation, age, diagnosis methods and management. STUDY DESIGN: historical cohort. METHOD: Patients with PV treated in Otolaryngology Department from HC-FMUSP between 1990 and 2001. RESULTS: From 23 patients, 91,3% were women and 8,7% men (p <0,01), proportion of 9:1. The age of diagnosis varied between 26 and 80 years, with average of 53 ± 18,4 years. In males, PV begun 24,6 ± 5,9 years earlier than in females (p=0,026). The most frequently sign in physical examination were bullous lesions in oral mucosa. Biopsy was used in 95% of the cases, direct immunofluorescence in 17,4% and indirect 8,7%. Prednisona was the therapeutic option in 78% of the patients, while deflazacort was used in 22%. Dapsona was associated in 4 cases. We obtained control of the disease in 74% of the cases and loss of the continuation after partial improvement in more 13% of the patients. We observed larger difficulty in disease control in males (p=0,04). CONCLUSIONS: The females prevailed over males. The age of diagnosis was higher in women. The treatment of choice was prednisona. PV was more easily controlled in females.
pemphigus; diagnosis; outcome assessment
