Pemphigus vulgaris: a 11-year review.
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.


CONCLUSIONS
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).
Chmurova
N
N
Department of Dermatovenereology, Faculty of Medicine, Comenius University, Bratislava, Slovakia. nada.chmurova@centrum.sk
Svecova
D
D
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D055815:Young Adult