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 .