Acta Demato-Venereologica 98-3CompleteContent | Page 23

380 SHORT COMMUNICATION

Advances in dermatology and venereology Acta Dermato-Venereologica

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

Jeffrey LOGET 1, Coralie BARBE 2, Sophie DUVERT-LEHEMBRE 3, Christophe BEDANE 4, Michaël MAIZIÈRES 1, Pascal JOLY 3, Amélie COURAUD 4, Julie PLÉE 1 and Philippe BERNARD 1

1

Department of Dermatology and 2 Clinical Research Unit, Reims University Hospital, FR-51092 Reims cedex, 3 Department of Dermatology, Rouen University Hospital, Rouen, 4 Department of Dermatology, Limoges University Hospital, Limoges, France. E-mail: pbernard @ chu-reims. fr Accepted Nov 13, 2017; Epub ahead of print Nov 14, 2017

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, Limousin and Haute-Normandie) encompassing a total population of 3.9 million. The aim of the centre is to improve healthcare, 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).

METHODS

1 https:// www. medicaljournals. se / acta / content / abstract / 10.2340 / 00015555-2848

All cases of AIBD, confirmed by cutaneous direct immunofluorescence( IF) microscopy analysis, were included. The data collected were demographic information( age, sex, home location), AIBD diagnosis and date, clinical characteristics at baseline( number and location of cutaneous or mucous blisters, other skin lesions), pattern and type of immune deposits observed by direct IF microscopy analysis, serum immunopathological investigations performed at time of diagnosis including standard indirect IF or indirect IF on salt-split skin, BP180 and BP230 enzyme-linked immunosorbent assay( ELISA) s, anti-desmoglein 1 and 3 ELI- SAs, and the initial type of management of patients( hospital or specialized outpatients’ clinics).

RESULTS

Table I. Demographic characteristics and diagnostic management of the main autoimmune bullous diseases( AIBD)

AIBD n

Age, years mean ± SD

Sex ratio F / M

Hospital-based management a n(%)

A total of 734 cases of AIBD were recorded, including 538 cases of BP( 73 %), 58 mucous membrane pemphigoid( MMP)( 8 %), 7 epidermolysis bullosa acquisita( EBA)( 1 %), and 79 pemphigus( 11 %)( 54 pemphigus vulgaris( PV), 23 pemphigus foliaceus( PF))( Table I). The number of cases recorded in each of the 3 reference centres was quite similar( 240, 234 and 260, respectively) with most of patients( n = 599, 82 %) living in the administrative region of the reference centre. Diagnostic and therapeutic management were initially performed at hospital in 540 cases( 74 %). At baseline, 253 patients with BP( 47 %) had extensive disease( i. e. more than 10 daily blisters) and 61( 11 %) had mucosal involvement, almost exclusively oral. Within MMP patients, oral involvement was present in 37 cases( 77 %), ocular in 20( 42 %), cutaneous in 30( 52 %) and genital in 8( 17 %). At diagnosis, the detection of serum autoantibodies was investigated by standard indirect IF in 307 cases( 42 %) and by ELISA measurement in 693 cases( 94 %)( Table I). Indirect IF on salt-split skin was performed at diagnosis in 504 cases of

IIF b n(%)

ELISA c n(%)

a Diagnosis report and treatment initially carried out in one of the departments of the referral centre. b Standard

indirect immunofluorescence performed at diagnosis. c Serum enzyme-linked immunosorbent assay for anti- BP180 and anti-BP230 auto-antibodies, or anti-desmoglein 1 and anti-desmoglein 3 auto-antibodies detection performed at diagnosis. SD: standard deviation; NA: not applicable.

BP( 94 %), in 55 cases of MMP( 95 %) and in all cases of EBA( n = 7).

Standardized incidence rates of all AIBD, of bullous pemphigoid( BP) and of pemphigus were calculated using the distribution by age group of the French population, both in the whole population and by sex. For standardized incidences rates, only patients living in the administrative region of the reference centre( n = 599) were considered. Based on the data in the Regibul Register, the annual standardized incidence rates were 24.9 cases per million people( 95 % doi: 10.2340 / 00015555-2848 Acta Derm Venereol 2018; 98: 380 – 381

This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2018 Acta Dermato-Venereologica.