Epidemiology project

Understanding who is affected, how many are affected, and the consequences of bullous pemphigoid is important, but hasn’t been looked at in the UK in over a decade. 

Electronic health records from a subset of English general practices and hospital records were used to identify people with bullous pemphigoid. We determined the number of people being diagnosed for the first time each year (incidence) and the number of people living with bullous pemphigoid each year (prevalence). We compared the deaths in people with bullous pemphigoid versus similar people without the disease.

Every year, about 8 people in every 100,000 are diagnosed with bullous pemphigoid for the first time. This number has been rising slowly over the last two decades. Bullous pemphigoid more commonly affects older people, particularly older men. In 2017, about 50 in every 100,000 adults were living with bullous pemphigoid. Focusing only on older people (>60 years), just over 1 in every 1,000 older person was living with bullous pemphigoid in 2017. People with bullous pemphigoid were more likely to die than similar people without the disease – three times more likely in the first two years after being diagnosed, and then 50% more likely following this. 

• This study has improved our understanding of bullous pemphigoid in England. Importantly, we have shown that bullous pemphigoid is more common in older people than what was previously thought and that it continues to be life-limiting. We hope that increased awareness of the disease and its associated mortality will improve the diagnosis and management of patients in the future. 

NIHR Research for Patient Benefit