The Regibul Register: A Tool for Monitoring the Distribution and Incidence of Autoimmune Bullous Dermatoses in Three French Regions, 2010 to 2015

Autoimmune bullous dermatoses (AIBD) are rare in Western countries (1). The French Rare Diseases Reference Centre was set up in 2005, and encompasses 3 university hospital departments (Reims, Limoges and Rouen) located in 3 distinct French regions (Champagne-Ardenne, Limou-sin and Haute-Normandie) encompassing a total population of 3.9 million. The aim of the centre is to improve health-care, research, nursing staff and patient information, and to provide national and international recommendations. In this context, a computerized database was prospectively created in 2010, giving rise to the Regibul Register. Data from all new cases of AIBD seen in the reference centre were collected using an anonymous standardized questionnaire (CNIL authorization no. 911 0021). Despite diagnosis and treatment advances, to date only a few studies have described the distribution and incidence of sub-epidermal AIBD (2-10, Table SI 1) and pemphigus (11, 12). Using data collected over a 6-year period (2010 to 2015) from the 3 reference centre sites, the aim was to describe clinical characteristics along with the initial management of included patients (primary objective), and to estimate both the distribution and standardized incidence rates of these AIBD after exclusion of patients who were not living in our 3 regions (secondary objective).