Pemphigus vulgaris: a 11-year review

Abstract

Background: Pemphigus vulgaris (PV) is a rare, chronic life-threatening autoimmune blistering disease of the skin and mucous membranes.

Methods: A retrospective analysis of 31 patients with the diagnosis of pemphigus vulgaris, admitted for hospitalization from January 1996 to December 2006. Descriptive statistics has been used for data evaluation.

Results: The average age at onset was 49.0 +/- 16.2 years, with female to male ratio 1.4/1. Diagnosis was confirmed histologically and by a direct immunofluorescence. Mucocutaneous form of PV was observed in 25 patients (80.%), mucous form in 3 patients (9.7%), cutaneuos form in 3 patients (9.7%). Factors preceding the onset of PV were most often viral (38.7%) and bacterial (35.5%) infections, dental focuses (25.8%), stress (16.1%), and contact with chemical substances (16.1%). 6 patients (19.4%) did not indicate any triggering factor. Corticosteroids alone were given to 18 patients, combined with azatioprin or cyclosporin to 13 patients, and 1 patient was treated with intravenous immunoglobulin. Adverse events were mostly osteopenia/osteoporosis (41.9%), hyperlipoproteinemia (41.9%), cataract (32.3%) and Cushing's syndrome (32.3%). Cutaneous and mucous infections were most often caused by Staphylococcus aureus and Candida albicans, respectively. 3 patients died (9.7%), and in 3 patients (9.7%) in a long term remission, the immunosuppressive treatment was discontinued.

Conclusion: Pemphigus vulgaris is still a life-threatening disease. Although corticosteroids dramatically improved the mortality, and are still considered the first-choice therapy, significant morbidity of the disease and the corticosteroid treatment still exists. The combination of corticosteroids with corticosteroid-sparing agents delays the onset of adverse events (Fig. 2, Ref. 33).