Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

Abstract

Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study Design Retrospective historical cohort study.Setting Computerised medical records from the health improvement network, a large population based UK general practice database.Participants Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls.Main outcome measures Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation.Results 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).Conclusions Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for in incidence are not clearly understood but have implications for identifying causative factors. are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

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

“…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). Th e reported incidence varies greatly among diff erent countries: from 0.25/100.000 inhabitants per year in Romania (6), through 2.2/100 000 inhabitants in France or 2.4/100.000 persons per year in the United States (8), to 4.3/100 000 persons per year in the United Kingdom (9). Th e incidence appears to be increasing in the last two decades (8,9), which may be attributed to greater proportion of older persons in the population (9), or to increasing incidence of neurological diseases, and use of certain medications which are implicated as risk factors for development of BP (10).…”

GM-CSF Modulates Autoantibody Production and Skin Blistering in Experimental Epidermolysis Bullosa Acquisita

“…In experimental autoimmune encephalomyelitis, GM-CSF is required to sustain neuroinflammation via myeloid cells infiltrating the CNS (14). Given the rising incidence of autoimmune diseases in general and autoimmune bullous dermatoses specifically (53,54), the so far limited therapeutic options (55,56), and the high morbidity and mortality of the patients (54), there is a clear need for novel treatment strategies. In several autoimmune diseases, such as rheumatoid arthritis, inhibition of cytokines or growth factors has dramatically improved the management of these conditions (57)(58)(59)(60)(61).…”

Oral mucosal lesions in patients with pemphigus and pemphigoid skin diseases: a cross sectional study from southern India.

“…Lan--gan et al in 2008, mentioned that mortality rate of pemphigus may range as 4.8% and 54%. 10 Oral mu--cosal manifestation (fluid filled blisters or bullous, erosions) was observed in all the patients, and most prevalent oral site was buc--cal mucosa (92.85%), which is fol--lowed by Soft palate (71.42%), lower lip (42.85%), tongue (35.71%), labial mucosa (28.57%), floor of mouth (21.42%), upper lip (14.28%), gingival (14.28%), and hard palate (7.14%). 78.57% (11 out of 14) patients had reported that, oral mucosal manifestation as a primary site of disease pro--cess.…”