C Boulard1, S Duvert Lehembre1, C Picard-Dahan2, J S Kern3, G Zambruno4, C Feliciani5, B Marinovic6, P Vabres7, L Borradori8, C Prost-Squarcioni9, B Labeille10, M A Richard11, S Ingen-Housz-Oro12, E Houivet13, V P Werth14, D F Murrell15, M Hertl16, J Benichou13, P Joly1. 1. Department of Dermatology, Rouen University Hospital, and INSERM U905, Institute for Research and Innovation in Biomedicine, University of Rouen, Rouen, France. 2. Department of Dermatology, Bichat University Hospital, AP-HP, Paris, France. 3. Department of Dermatology, Medical Center, University of Freiburg, Freiburg, Germany. 4. Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IRCCS, Via Monti di Creta 104, 00167, Roma, Italy. 5. Policlinico Gemelli - Università Cattolica del 'Sacro Cuore', Largo Gemelli 8, 00168, Roma, Italy. 6. University Hospital Center Zagreb, Department of Dermatology and Venereology, Salata 4, 10000, Zagreb, Croatia. 7. Department of Dermatology, Dijon University Hospital, Dijon, France. 8. Department of Dermatology, Universitätsklinik für Dermatologie Inselspital, CH-3010, Bern, Switzerland. 9. Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France. 10. Department of Dermatology, Saint Etienne University Hospital, Saint Etienne, France. 11. 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. 12. Department of Dermatology, Henri Mondor University Hospital, AP-HP, Créteil, France. 13. Department of Biostatistics, INSERM U657, Rouen University Hospital, University of Rouen, Rouen, France. 14. Department of Dermatology, University of Pennsylvania, and Philadelphia VAMC, Philadelphia, PA, U.S.A. 15. Department of Dermatology, St George Hospital, University of NSW, Sydney, Australia. 16. Department of Dermatology, Philipps University Marburg, Marburg, Germany.
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. OBJECTIVES: 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.
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. OBJECTIVES: 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: Dedee F Murrell; Sandra Peña; Pascal Joly; Branka Marinovic; Takashi Hashimoto; Luis A Diaz; Animesh A Sinha; Aimee S Payne; Maryam Daneshpazhooh; Rüdiger Eming; Marcel F Jonkman; Daniel Mimouni; Luca Borradori; Soo-Chan Kim; Jun Yamagami; Julia S Lehman; Marwah Adly Saleh; Donna A Culton; Annette Czernik; John J Zone; David Fivenson; Hideyuki Ujiie; Katarzyna Wozniak; Ayşe Akman-Karakaş; Philippe Bernard; Neil J Korman; Frédéric Caux; Kossara Drenovska; Catherine Prost-Squarcioni; Snejina Vassileva; Ron J Feldman; Adela Rambi Cardones; Johann Bauer; Dimitrios Ioannides; Hana Jedlickova; Francis Palisson; Aikaterini Patsatsi; Soner Uzun; Savas Yayli; Detlef Zillikens; Masayuki Amagai; Michael Hertl; Enno Schmidt; Valeria Aoki; Sergei A Grando; Hiroshi Shimizu; Sharon Baum; Guiseppe Cianchini; Claudio Feliciani; Pilar Iranzo; Jose M Mascaró; Cezary Kowalewski; Russell Hall; Richard Groves; Karen E Harman; M Peter Marinkovich; Emanual Maverakis; Victoria P Werth Journal: J Am Acad Dermatol Date: 2018-02-10 Impact factor: 11.527
Authors: D M Chen; A Odueyungbo; E Csinady; L Gearhart; P Lehane; M Cheu; M Maho-Vaillant; C Prost-Squarcioni; V Hebert; E Houivet; S Calbo; F Caillot; M L Golinski; B Labeille; C Picard-Dahan; C Paul; M A Richard; J D Bouaziz; S Duvert-Lehembre; P Bernard; F Caux; M Alexandre; S Ingen-Housz-Oro; P Vabres; E Delaporte; G Quereux; A Dupuy; S Debarbieux; M Avenel-Audran; M D'Incan; C Bedane; N Bénéton; D Jullien; N Dupin; L Misery; L Machet; M Beylot-Barry; O Dereure; B Sassolas; J Benichou; P Musette; P Joly Journal: Br J Dermatol Date: 2019-11-28 Impact factor: 9.302