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. © 2016 British Association of Dermatologists
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 / Boulard, ; and Duvert Lehembre, C. a.; and Picard-Dahan, S. a.; and Kern, C. b.; and Zambruno, J. S. c.; D., G.; Feliciani, C.; and Marinovic, C. e.; and Vabres, B. f.; and Borradori, P. g.; and Prost-Squarcioni, L. h.; and Labeille, C. i.; and Richard, B. j.; and Ingen-Housz-Oro, M. A. k.; and Houivet, S. l.; and Werth, E. m.; and Murrell, V. P. n.; and Hertl, D. F. o.; and Benichou, M. p.; and Joly, J. m.; and the International Pemphigus Study Groupq and Hofmann, P. a.; and Camaioni, S. q.; and Didona, B. q.; and Fania, B. q.; and Della Torre, L. q.; and Caux, R. q.; and Alexandre, F. q.; and Paul, M. q.; and Bulai Livideanu, C. q.; and Delaporte, C. q.; and Dupuy, E. q.; and Avenel-Audran, A. q.; and Schmidt, M. q.; and Schumacher, E. q.; and Bagot, N. q.; and Tancrede-Bohin, M. q.; and Bouaziz, E. q.; and D'Incan, J. D. q.; and Quereux, M. q.; and Skowron, G. q.; and Beylot-Barry, F. q.; and Doutre, M. q.; and Bedane, M. S. q.; and Bernard, C. q.; and Machet, P. q.; and Jullienq and Debarbieux, L. q.; and Dereure, S. q.; and Hünefeld, O. q.; and Schanz, C. q.; and Franck, S. q.; and Maillard, N. q.; and Misery, H. q.; and Sassolas, L. q.; and Abasq, B. q.; and Bouyeure, C. q.; and Lagoutte, A. C. q.; and Ferranti, P. q.; Q., V.. - In: BRITISH JOURNAL OF DERMATOLOGY. - ISSN 0007-0963. - 175:1(2016), pp. 142-149. [10.1111/bjd.14405]
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
c. FelicianiMembro del Collaboration Group
;
2016-01-01
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. © 2016 British Association of DermatologistsI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
