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. Boulard; S. Duvert Lehembre; C. Picard-Dahan; J.S. Kern; G. Zambruno; C. Feliciani; B. Marinovic; P. Vabres; L. Borradori; C. Prost-Squarcioni; B. Labeille; M.A. Richard; S. Ingen-Housz-Oro; E. Houivet; V.P. Werth; D.F. Murrell; M. Hertl; J. Benichou; P. Joly

Disclosures

The British Journal of Dermatology. 2016;175(1):142-149. 

In This Article

Abstract and Introduction

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.

Introduction

Pemphigus is a rare autoimmune disease that causes skin and mucosal blistering and erosions.[1] Two major types of pemphigus have been described: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). A systematic review of outcome measures for assessing pemphigus severity recently identified 116 different measurement systems, which were reported in 96 articles published over the last 25 years.[2] Most studies used outcome measures based on global scales such as Physician's Global Assessment (PGA), or nonspecific ratings of disease activity encompassing lesion count and complete healing, or scoring systems based on semiquantitative evaluation of cutaneous and/or mucosal involvement as proposed, respectively, by Harman et al.,[3] Ikeda et al.,[4] Agarwal et al.[5] and Chams-Davatchi et al.[6] The lack of uniform measurement systems has constituted a major drawback for comparing different therapeutic regimens. Additionally, there is a great need for a validated and well-accepted scoring system to help physicians in clinical practice to make therapeutic choices and adapt treatment according to disease activity.

Recently, two new scoring systems have been proposed to provide an objective measure of disease activity in pemphigus:[7] the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), developed by German dermatologists,[8] and the Pemphigus Disease Area Index (PDAI), developed by an international panel of experts.[9] They both quantify the extent of cutaneous and mucosal erosions, as well as either a measure of patient discomfort in the ABSIS score or a measure of skin damage in the PDAI score. The use of these scores in clinical practice is limited by the absence of cut-off values to allow differentiation between moderate, significant and extensive types of pemphigus. This distinction is important in clinical practice to propose adequate treatment, and in clinical trials to allow meaningful comparisons between them.

The aim of this study was to propose cut-off values based on the ABSIS and PDAI scoring systems, in order to define three categories of pemphigus activity, namely moderate, significant and extensive forms.

Comments

3090D553-9492-4563-8681-AD288FA52ACE

processing....