Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Jun;184(6):1106-1112.
doi: 10.1111/bjd.19611. Epub 2020 Nov 29.

International validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid

W Masmoudi  1 , M Vaillant  2 , S Vassileva  3 , A Patsatsi  4 , G Quereux  5 , C Moltrasio  6 , C Abasq  7 , C Prost-Squarcioni  8 , D Kottler  9 , D Kiritsi  10 , N Litrowski  11 , P Plantin  12 , L Friedrichsen  13 , A Zebrowska  14 , S Duvert-Lehembre  15 , S Hofmann  16 , V Ferranti  1 , F Jouen  1   2 , P Joly  1   2 , V Hebert  1   2 ; EADV Autoimmune Bullous Skin Disease Task Force
Affiliations

International validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid

W Masmoudi et al. Br J Dermatol. 2021 Jun.

Abstract

Background: The Bullous Pemphigoid Disease Area Index (BPDAI) score has been proposed to provide an objective measure of bullous pemphigoid (BP) activity.

Objectives: The objective of this study was to calculate BPDAI cut-off values defining mild, moderate and severe BP. We also aimed to assess the interrater reliability and correlation with the number of daily new blisters, and anti-BP180 and anti-BP230 antibodies.

Methods: Severity scores were recorded by two blinded investigators. Anti-BP180 and anti-BP230 antibodies were measured using an enzyme-linked immunosorbent assay (ELISA). Cut-off values defining mild, moderate and severe subgroups were calculated based on the 25th and 75th percentiles of the BPDAI score.

Results: In total, 285 patients with BP were enrolled from 50 dermatology departments in Europe. Median BPDAI activity was 37·5 points (range 0-164). Cut-off values corresponding to the first and third quartiles of the BPDAI score were 20 and 57, respectively; thus, these values were used to define mild (≤ 19), moderate (≥ 20 and ≤ 56) and severe (≥ 57) BP. The median BPDAI score for patients with ≤ 10 daily new blisters was 26 [interquartile range (IQR) 17-45], and for patients with > 10 daily new blisters the median score was 55 (IQR 39-82). The BPDAI intraclass correlation coefficient measured at baseline was 0·97 and remained higher than 0·90 up to month 6. The improvement in the BPDAI score was correlated with the absolute decrease in anti-BP180 ELISA value (Spearman's rank r = 0·34, P < 0·004), but not with anti-BP230 antibodies (r = 0·17, P = 0·15).

Conclusions: This study suggests cut-off values of 20-57 for BPDAI to distinguish mild, moderate and severe BP, and confirms that it is a robust tool to assess BP severity precisely.

PubMed Disclaimer

Comment in

References

    1. Bernard P, Vaillant L, Labeille B et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131:48-52.
    1. Zillikens D, Wever S, Roth A et al. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 1995; 131:957-8.
    1. Gudi VS, White MI, Cruickshank N et al. Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland. Br J Dermatol 2005; 153:424-7.
    1. Joly P, Baricault S, Sparsa A et al. Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 2012; 132:1998-2004.
    1. Langan SM, Smeeth L, Hubbard R et al. Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study. BMJ 2008; 337:a180.