Pemphigus vulgaris: a clinical study of 44 cases over a 20-year period.

Background: Pemphigus vulgaris (PV) is a rare, life-threatening, autoimmune bullous disease with unpredictable progression and prognosis.

Objectives: This study aimed to analyze long-term outcomes in patients with PV, focusing on factors that may influence the prognosis of the disease, including the phenotype of PV, age at onset, multiplicity of mucosal involvement, relapse and remission rates, and survival functions.

Methods: A retrospective analysis was conducted in 44 patients who were diagnosed and monitored from 1993 to 2012. Statistical analyses were conducted using the Mann-Whitney test and Kruskal-Wallis test. Survival data were analyzed using Kaplan-Meier and log rank tests. Fisher's exact test was used to assess statistical significance at P < 0.05.

Results: A total of 79.5% of patients were identified as having mucocutaneous PV. The female : male gender ratio was 1.58; in severe PV, this increased to 2.8. The difference between the genders in age at onset was statistically significant (P = 0.05). Furthermore, multiple mucosal involvements in the oral cavity and at other sites were associated with severe mucocutaneous PV. Multiple relapses occurred more frequently in patients with severe PV than in those with mild PV (P = 0.001). The remission survival of females was longer than that of males (P = 0.003).

Conclusions: Poor prognosis factors include the severe mucocutaneous type of PV, especially in female patients, and multiple mucosal involvements in the oral cavity and other mucosal sites. Female patients were younger than male patients and were more often observed to have severe mucocutaneous PV and to have a higher median period of remission survival than male patients.