Bullous pemphigoid and neurological disease: statistics from a dermatology service

Abstract

Bullous pemphigoid (BP) is an autoimmune, acquired, cutaneous disease caused by the production of autoantibodies against hemidesmosomes' components in the basement membrane. The estimated incidence in Europe ranges from 7 to 43 cases per million inhabitants per year. Several studies have reported an association between BP and neurological disorders (ND). Our cohort of Bullous pemphigoid and ND is the first in Brazil and showed a significantly high prevalence of neurological and/or psychiatric diseases, especially cerebrovascular accident (CVA) and dementia, in agreement with the prevalence reported in several studies published in the medical literature in recent years.

Neurological disorders are associated with bullous pemphigoid

“…In part, this may be due to the age of the BP patients, which in recent studies ranged from 64 to 83 years. [14][15][16]18,19,21,25 We observed that dementia was the most prevalent neurological disorder in patients with BP (32%). This association is in line with Kwan et al 20 who conducted a retrospective case-control study on 43 patients with BP and reported that dementia was the only neurological disorder that had any statistically significant association (P = 0.002) with this autoimmune disease.…”

“…It has previously been shown that the incidence of cerebrovascular accident (CVA) and dementia in patients with BP is significantly elevated. 18 Tarazone et al 19 also revealed a significantly greater prevalence of neurological disorders, especially CVA and dementia, in patients with BP. With 9%, stroke was the second most common neurological disorder in patients with BP, with a highly statistically significant association (P = 0.0015).…”

“…11,12 There is growing evidence to suggest an association between BP and a range of neurological disorders including dementia, cerebral stroke, Parkinson's disease, cerebrovascular disease, multiple sclerosis, epilepsy and polyneuropathies. [13][14][15][16][17][18][19][20][21][22] While the immunological mechanisms have not been fully elucidated, the association between BP and neurological disorders may be due to immune responses arising from cross-reactivities of skin autoantibodies with BP antigens or their isoforms that are known to be expressed in brain and neuronal tissue. 8,10,14,23 In our study, we aimed to analyse the clinical characteristics of patients with BP and to assess the relationship between BP and neurological disorders.…”

Localized Bullous Pemphigoid on the Site of Knee Arthroplasty: A Case Report

“…Bullous pemphigoid is an acquired, autoimmune disease confi ned to the skin, characterized by formation of autoantibodies which are directed towards components of hemidesmosomes in the basement membrane (5). It is the most common autoimmune bullous disease in Western Europe and North America (6), and probably in most countries of the world (5), which aff ects mostly the elderly (7).…”

A multi‐hit hypothesis of bullous pemphigoid and associated neurological disease: Is HLA‐DQB1*03:01, a potential link between immune privileged antigen exposure and epitope spreading?

“…This association has been studied in case‐control, case series, and large population based studies. These studies have spanned extensive and diverse geographical territories including North America, South America, Western Europe, Eastern Europe, and Asia . The prevalence of neurologic disease is significantly greater in patients with BP when compared with control populations, and it is estimated to be between 28% and 56% according to multiple studies .…”