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.

[Tuberculosis, primary biliary cirrhosis and autoimmunity (apropos of a case in Tunisia)]. / Tuberculose, cirrhose biliaire primitive et autoimmunité (a propos d'un cas tunisien).

We report the case of a 70 year-old Tunisian patient who developed antimitochondrial antibodies and anti-ADN during urogenital tuberculosis with clinical and biological signs of primary biliary cirrhosis and systemic lupus erythematosus. We discuss the association of the three diseases and the etiopathogeny of the autoimmune mechanisms induced by Mycobacterium tuberculosis.