Epidemiologic analysis and clinical course of 84 consecutive cases of pemphigus in eastern Sicily

Background The purpose of this study was to examine the clinical and epidemiologic features of pemphigus in eastern Sicily.
Methods All new cases of pemphigus seen in the Dermatology Clinic at the University of Catania during the period January 1982 to June 1996 were studied retrospectively.
Results Eighty-four patients with pemphigus represented 1.3% of 6653 patients admitted to our clinic in a 13.5-year period, with an average annual incidence of six cases per year. The most common form of presentation was pemphigus vulgaris (PV) (75%), followed by pemphigus erythematosus (17%), pemphigus herpetiformis (6%), and pemphigus vegetans (2%). In 6% of PV patients, the oral lesions were not followed by skin involvement, and the lag time between the onset of illness and the final diagnosis in five PV cases was prolonged, ranging from 4 to 7 years, probably due to the mild clinical presentation of the disease. Two trauma-related PV cases were documented; in one case, the blisters were located in a surgical scar and in the other in a burn scar.
Conclusions Our findings showed that pemphigus has a relatively high prevalence in eastern Sicily compared with other Italian regions; PV is the most common variant, and may have a mild course not always requiring aggressive therapy.