Background Pemphigus vulgaris is a chronic autoimmune mucocutaneous blistering disease. Only a few studies have evaluated the epidemiological and aetiological parameters of pemphigus vulgaris in a large group of patients over the long term.
Methods The sample included 155 patients with a diagnosis of pemphigus who attended the pemphigus clinic of a major tertiary medical centre from 1976 to 2004. Data were obtained from the patient files and entered into an ad hoc form; patients were contacted by telephone for missing information.
Results The female-to-male ratio was 1.5 : 1. Non-Ashkenazi Jews accounted for 37% of the sample. In only 10% of the patients was a potential aetiologic or precipitating factor identified.
Conclusions Pemphigus vulgaris is characterized by a female predominance, consistent with other autoimmune disease. The gender, age and ethnic distribution of affected patients have not changed in the last 40 years. In the vast majority of cases, the aetiologic or precipitating factor is unknown, although drugs appear to be very rare.