Incidence and Mortality of Bullous Pemphigoid in France

Journal of Investigative Dermatology (2012) 132, 1998–2004; doi:10.1038/jid.2012.35; published online 15 March 2012

Incidence and Mortality of Bullous Pemphigoid in France

Pascal Joly1, Sophie Baricault1, Agnès Sparsa2, Philippe Bernard3, Christophe Bédane2, Sophie Duvert-Lehembre1, Philippe Courville4, Pierre Bravard5, Brigitte Rémond6, Valérie Doffoel-Hantz2 and Jacques Bénichou7

1. 1Department of Dermatology, INSERM U905, Rouen University Hospital, University of Rouen, Rouen, France
2. 2Department of Dermatology, Dupuytren Hospital, University of Limoges, Limoges, France
3. 3Department of Dermatology, Robert Debré Hospital, University of Reims, Reims, France
4. 4Department of Pathology, Rouen University Hospital, University of Rouen, Rouen, France
5. 5Department of Dermatology, Le Havre General Hospital, Le Havre, France
6. 6Department of Dermatology, Évreux General Hospital, Évreux, France
7. 7Department of Biostatistics, INSERM U657, Rouen University Hospital, University of Rouen, Rouen, France
Correspondence: Pascal Joly, Clinique Dermatologique, Rouen University Hospital, 1 Rue de Germont, 76031 Rouen Cedex, France. E-mail: [email protected]

Received 9 August 2011; Revised 15 December 2011; Accepted 22 December 2011
Advance online publication 15 March 2012

Abstract

A major increase in the incidence of BP has been recently reported in the United Kingdom. In addition, there are some controversies about the over-mortality of BP patients. The primary objective was to reevaluate the incidence of BP in France as compared with that we estimated 15 years ago. The secondary objective was to assess mortality of BP patients. BP incidence was retrospectively estimated from all BP cases diagnosed between January 2000 and December 2005 in three French regions with a total population of 3.858 million inhabitants. BP mortality was assessed from a prospective cohort accrued during the same time period. A total of 502 incident BP patients (mean age: 82.6±8.8years) were identified. Overall estimated incidence was 21.7 cases per million persons per year (95%CI:19.8–23.7 cases per million persons per year), which is about 3-fold higher than the incidence that we estimated 15 years ago. In the population aged 70 years or above, BP incidence was 162 cases per million per year (95%CI:147–177 cases per million per year). The overall 1-year survival rate was 62% (95% CI: 56–67%). The risk of death for BP patients was more than six times greater than that for the general population (SMR:6.60; 95%CI:5.47–7.90). The incidence of BP in France has increased 3-fold in the last 15 years. BP is associated with high mortality.