title: Calculation of cut-off values based on the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus.
abstract: BACKGROUND
  Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus.
  
  
  OBJECTIVE
  To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores.
  
  
  METHODS
  In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups.
  
  
  RESULTS
  Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P < 0·001), PDAI (P < 0·001), PGA (P < 0·001) and DLQI (P = 0·03) scores.
  
  
  CONCLUSIONS
  This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus.
authors.affiliation: Department of Dermatology, Rouen University Hospital, and INSERM U905, Institute for Research and Innovation in Biomedicine, University of Rouen, Rouen, France.
authors.forename: C
authors.initials: C
authors.lastname: Boulard
authors.affiliation: Department of Dermatology, Rouen University Hospital, and INSERM U905, Institute for Research and Innovation in Biomedicine, University of Rouen, Rouen, France.
authors.forename: S
authors.initials: S
authors.lastname: Duvert Lehembre
authors.affiliation: Department of Dermatology, Bichat University Hospital, AP-HP, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Picard-Dahan
authors.affiliation: Department of Dermatology, Medical Center, University of Freiburg, Freiburg, Germany.
authors.forename: J S
authors.initials: JS
authors.lastname: Kern
authors.affiliation: Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IRCCS, Via Monti di Creta 104, 00167, Roma, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Zambruno
authors.affiliation: Policlinico Gemelli - Università Cattolica del 'Sacro Cuore', Largo Gemelli 8, 00168, Roma, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Feliciani
authors.affiliation: University Hospital Center Zagreb, Department of Dermatology and Venereology, Salata 4, 10000, Zagreb, Croatia.
authors.forename: B
authors.initials: B
authors.lastname: Marinovic
authors.affiliation: Department of Dermatology, Dijon University Hospital, Dijon, France.
authors.forename: P
authors.initials: P
authors.lastname: Vabres
authors.affiliation: Department of Dermatology, Universitätsklinik für Dermatologie Inselspital, CH-3010, Bern, Switzerland.
authors.forename: L
authors.initials: L
authors.lastname: Borradori
authors.affiliation: Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France.
authors.forename: C
authors.initials: C
authors.lastname: Prost-Squarcioni
authors.affiliation: Department of Dermatology, Saint Etienne University Hospital, Saint Etienne, France.
authors.forename: B
authors.initials: B
authors.lastname: Labeille
authors.affiliation: Aix-Marseille University, UMR 911, INSERM CRO2, 'Centre de Recherche en Oncologie Biologique et Oncophamacologie', Department of Dermatology, Hôpital Timone, Assistance Publique des Hôpitaux de Marseille, 264 Rue Saint Pierre, 13385, Marseille, France.
authors.forename: M A
authors.initials: MA
authors.lastname: Richard
authors.affiliation: Department of Dermatology, Henri Mondor University Hospital, AP-HP, Créteil, France.
authors.forename: S
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Department of Biostatistics, INSERM U657, Rouen University Hospital, University of Rouen, Rouen, France.
authors.forename: E
authors.initials: E
authors.lastname: Houivet
authors.affiliation: Department of Dermatology, University of Pennsylvania, and Philadelphia VAMC, Philadelphia, PA, U.S.A.
authors.forename: V P
authors.initials: VP
authors.lastname: Werth
authors.affiliation: Department of Dermatology, St George Hospital, University of NSW, Sydney, Australia.
authors.forename: D F
authors.initials: DF
authors.lastname: Murrell
authors.affiliation: Department of Dermatology, Philipps University Marburg, Marburg, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Hertl
authors.affiliation: Department of Biostatistics, INSERM U657, Rouen University Hospital, University of Rouen, Rouen, France.
authors.forename: J
authors.initials: J
authors.lastname: Benichou
authors.affiliation: Department of Dermatology, Rouen University Hospital, and INSERM U905, Institute for Research and Innovation in Biomedicine, University of Rouen, Rouen, France.
authors.forename: P
authors.initials: P
authors.lastname: Joly
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis*; D011446:Prospective Studies; D012016:Reference Values; D012720:Severity of Illness Index; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis*