Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid

Abstract

Autoimmune bullous diseases are rare, organ-specific, a group of blistering disease of skin and mucous membranes. Recent studies suggest that the frequency of the autoimmune bullous diseases has been increasing. Pemphigus vulgaris and bullous pemphigoid are the most frequently reported autoimmune bullous diseases. High incidence of autoimmune bullous diseases in some ethnic groups such as pemphigus in Ashkenazi Jewish, or in some regions such as pemphigus foliaceus in Brazil has been shown to be related to genetic and environmental factors, respectively. Pemphigus has been reported more frequently in the female gender. Although it is most frequently diagnosed between the ages 50 and 60 in European countries, in the remaining countries in the world, it is seen between the ages of 30 and 50. Bullous pemphigoid is generally seen above 70 years of age. Although overall incidence is slightly higher in females, after the age of 80 years it is more frequent in males. Both pemphigus vulgaris and bullous pemphigoid has a chronic course with recurrences. Mortality risk of the patients with bullous pemphigoid was found at least 2 times higher and the mortality risk of the patients with pemphigus was found approximately 3 times higher than that of the general population. In this review, the results obtained from the epidemiological studies were analyzed according to geographic regions, and especially epidemiologic features of two prevalent autoimmune bullous diseases, pemphigus and bullous pemphigoid have been discussed.

Epidemiological and clinical aspects of pemphigus in Senegal

“…Cependant, leurs caractéristiques épidémiologiques et cliniques restent très variables selon les régions du monde et selon les groupes ethniques. Ainsi, le Pemphigus Foliacé (PF) demeure endémique en Amérique du Sud et en Tunusie, alors que le pemphigus herpétiforme est plus décrit en Europe de l'Est [1,2]. Cette disparité serait probablement liée à des facteurs génétiques et environnementaux.…”

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

“…PV is the most commonly seen and representative clinical form of pemphigus with an incidence of 0.1-0.5/100.000 population [1,2,7,13]. The average age at onset is usually at fourth and fifth decades, but may occur in the elderly or children.…”

“…The average age at onset is usually at fourth and fifth decades, but may occur in the elderly or children. The incidence rate is higher among patients with Jewish and Mediterranean ancestry [1,2,5,7,13]. Various environmental factors such as drugs (captopril, penicillamine), infections (herpes simplex virus, Epstein-Barr virus, etc.…”

“…PF has a universal occurrence and occurs sporadically, while the endemic form of PF, called as fogo selvagem (FS) or wild fire (WF), is predominantly seen in the rural and tropical regions of Brazil [5,7,16,58,59]. Another variant of PF, a localized form, is called as pemphigus erythematosus (PE) [16,59].…”

“…It is a chronic potentially life-threating bullous disorder if not treated on time [4,7,8]. The phenotypes of pemphigus represent a complex spectrum with multiple genetic and environmental factors playing a role in disease pathogenesis [9,10].…”