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. 2008 Jul 9;337(7662):a180.
doi: 10.1136/bmj.a180.

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

S M Langan  1 , L Smeeth, R Hubbard, K M Fleming, C J P Smith, J West
Affiliations

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

S M Langan et al. BMJ. .

Abstract

Objective: To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom.

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 the changes in incidence are not clearly understood but have implications for identifying causative factors. Both disorders are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

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Conflict of interest statement

Competing interests: None declared.

Figures

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Fig 1 Age at diagnosis in patients with bullous pemphigoid and pemphigus vulgaris
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Fig 2 Kaplan-Meier curve for bullous pemphigoid, showing mortality over time since diagnosis or “pseudodiagnosis” and number of patients remaining in study
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Fig 3 Kaplan-Meier curve for pemphigus vulgaris, showing mortality over time since diagnosis or “pseudodiagnosis” and number of patients remaining in study
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Fig 4 Age adjusted rates of bullous pemphigoid and pemphigus vulgaris, with direct standardisation to European standard population
See this image and copyright information in PMC

Comment in

References

    1. Naldi L, Bertoni M, Cainelli T. Feasibility of a registry of pemphigus in Italy: two years experience. Int J Dermatol 1993;32:424-7. - PubMed
    1. Adam BA. Bullous diseases in Malaysia: epidemiology and natural history. Int J Dermatol 1992;31:42-5. - PubMed
    1. Garcia-Doval I, Mayo E, Nogueira Farina J, Cruces MJ. Bullous pemphigoid triggered by influenza vaccination? Ecological study in Galicia, Spain. Br J Dermatol 2006;155:820-3. - PubMed
    1. Jung M, Kippes W, Messer G, Zillikens D, Rzany B. Increased risk of bullous pemphigoid in male and very old patients: a population-based study on incidence. J Am Acad Dermatol 1999;41:266-8. - PubMed
    1. Gudi VS, White MI, Cruickshank N, Herriot R, Edwards SL, Nimmo F, et al. Annual incidence and mortality of bullous pemphigoid in the Grampian region of north-east Scotland. Br J Dermatol 2005;153:424-7. - PubMed

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