Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study.

Background: Pemphigus vegetans (P Veg) is a rare clinical form of pemphigus. Studies on P Veg are rare in the literature and none has so far evaluated the prognostic parameters.

Objective: In this retrospective study of P Veg, we aimed to analyse epidemiological, clinical, immunopathological and therapeutic data. Study of prognostic factors with accuracy of patient survival was also carried out.

Methods: This is a retrospective study (1981-2009) including 17 cases of P Veg. Statistical analysis was performed with chi-square and Fisher tests looking for a possible relationship between clinical data and prognostic factors. Follow-up time and disease-free survival time were estimated using Kaplan-Meier methods. Clinical data were evaluated in univariate analysis looking for a significant association with survival. Equality of survival distribution was studied using log rank test.

Results: The hospital prevalence of P Veg was 0.084‰ with a frequency of 9.1% among pemphigus. The mean age at onset was 47.6 years, with a sex-ratio (F/M) about 4.66. Neumann P Veg was the predominant clinical form (11/17). Clinically, the lesions were multifocal (16/17), prevailed on folds and mucous membranes. Under corticosteroids the mean period for healing was 24 ± 9 days. During the follow-up time, three patients died and 11 patients relapsed. Median of overall relapse-free survival was 13 ± 1.7 months. No significant association between clinical data and prognostic factors was found.

Limitations: This study was a retrospective chart analysis and the number of patients was small.

Conclusion: The P Veg seems to be more frequent in Tunisia with high rate of mortality.