Epidemiology of pemphigus in Macedonia: a 15-year retrospective study (1990-2004).

Background: Pemphigus is an autoimmune blistering skin disease mediated by auto-antibodies directed against desmoglein proteins. There are only a few epidemiological studies on pemphigus. Our objective was to determine the epidemiological features of pemphigus in Macedonia, and to compare the results with those reported elsewhere.

Methods: Diagnosis in all cases was confirmed by histopathology and direct immunofluorescence. Binomial distribution testing and Fisher's exact-test at the 0.01 level of significance were used to determine if particular demographic groups were over-/ under-represented among the pemphigus patients.

Results: One hundred and thirty-three new pemphigus cases were diagnosed in Macedonia from 1990-2004. The average annual incidence was 0.44/100,000 inhabitants (SD = 0.17). The incidence doubled to 0.89 in 2001 during the local armed unrest. The disease did not affect either gender to a greater extent. The average annual incidence was 0.51 for ethnic Macedonians. Roma (Gypsies) had a statistically significantly higher incidence of pemphigus at 2.4 cases/100,000 individuals. Ethnic Albanians had statistically significantly lower incidence of 0.1 cases/100,000 individuals. The most common variant was pemphigus vulgaris (77.4%).

Conclusions: The annual incidence for pemphigus in Macedonia is 0.44 cases/100,000 inhabitants. Most common form was pemphigus vulgaris. An epidemiological peak occurred in 2001 during the local armed conflict. Macedonian Roma had a sixfold higher incidence of pemphigus compared with the overall population; ethnic Albanians had a fourfold lower incidence.