High Risk of Death in Elderly Patients With Extensive Bullous Pemphigoid

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Abstract
BULLOUS pemphigoid (BP) is an acquired autoimmune blistering disorder that occurs mostly in people older than 65 years. This disease is not rare in the elderly. Its incidence has been estimated in France as 7 new cases per million person-years1 in adults, ie, an age-adjusted incidence greater than 50 cases per million person-years for people older than 65 years. Bullous pemphigoid is usually considered a benign and easily manageable disease with a "low mortality rate"2 and a prognosis "significantly better than that of pemphigus."3 These opinions, however, were not based on comparative data. In 2 classic articles,4,5 Savin studied the risk factors among patients who died of pemphigus or BP, as reported in death certificates. In these studies, most causes of death appeared to be related to corticosteroid therapy, but no data on death rates were provided. In published series, death rates varied from 6% at 2 years to 40% at 1 year.6-10 Such variations demonstrate that death rates in patients with BP have been poorly quantified but may be higher than expected in the elderly. The aim of our study was to estimate the death rate and to look for risk factors for death in patients with BP, taking advantage of homogeneous data collection on autoimmune blistering diseases in several centers in France.