Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality

Abstract

Little is known about differences in epidemiological features and prognosis between pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The objective of this study was to compare PV and PF patients regarding ethnic variations and mortality rates. Mortality of PV and PF patients was compared with age- and sex-matched control subjects in the general population. The study cohort comprised 207 patients with PV and 30 with PF diagnosed during the period 2000 to 2015. The incidence rate of PV among Jews was 3.6-fold higher than among Arabs (p<0.001), whereas no ethnic predisposition to PF was noted (p = 0.379). The risk of death for patients with PV was almost 3-fold higher than in the general population (standardized mortality ratio (SMR) 2.6). For patients with PF, the risk of mortality was not significantly increased relative to the general population (SMR 1.4). There is a racial predisposition to PV, whereas PF is sporadic. Mortality among patients with PV is higher compared with PF and the general population.

Incidence of pemphigus vulgaris and pemphigus foliaceus in North‐East Poland (Podlaskie Province) – a 15‐year (2001–2015) bicentric retrospective study

“…In our study, PV was diagnosed more frequently than PF, constituted two‐thirds of all cases of pemphigus group that resulted in higher incidence of PV than PF, which is in concordance with other European studies’ results . A higher proportion of PV (80% or more of all pemphigus cases) was found in Spain, Romania, Turkey, and Israel …”

“…We have found that age of PV patients was significantly lower than that of PF patients. Similarly older age of PF than PV was observed in Israel and in Spain . In contrast, in a study from Sicily, patients with PV were slightly older than those with pemphigus erythematosus – a variant of PF (56 vs. 54 years) .…”

“…Consequently, the incidence of pemphigus in women was significantly higher than in men. Only in a study from Israel was the incidence of PF higher in men than in women …”

“…In Europe, pemphigus affected females slightly more frequently than men . In only one study from Spain, authors found male to female ratio to be 1.22 .…”

“…Circulating autoantibodies were shown to be pathogenic in in vitro and in vivo studies . Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are two main clinical variants . Pemphigus herpetiformis, IgA pemphigus, pemphigus vegetans, or paraneoplastic pemphigus are less frequently seen variants .…”