Epidemiology

The prevalence of bullous pemphigoid in the US is reported as 6 to 10 cases per million, with a mean age of onset of 65 years.[2][5] A disease primarily of the aged, with an average onset of age 80 to 89 years; there is concern that the incidence may rise with the increased numbers of people living to advanced age.[6] One study in the UK reported a 17% average yearly increase in the incidence of bullous pemphigoid, while a study in France reported a threefold increase in incidence over a period of 15 years.[7][8]

Data on sex differences are conflicting.[6][9][10] Some studies suggest the incidence may be higher in women until age 75 years, after which the incidence is higher in men.[10] Rarely, the disorder is seen in children.[11][12]

In a 2-year retrospective study investigating all subepidermal immunobullous disorders seen at the National Skin Centre in Singapore, bullous pemphigoid accounted for 88% of all diagnoses, with predilection for ethnic Chinese and an average age of onset of 77 years.[13] North East Scotland appears to have a relatively high incidence of bullous pemphigoid (14 cases per million per year) when compared with incidence rates in continental Europe.[14] The cause of this variability is not clear and may be related to racial predisposition to autoimmune skin disease, prevalence of predisposing HLA genotypes in the study population, or environmental influence.

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