Skip to main content
Top
Published in: BMC Dermatology 1/2020

01-12-2020 | Common Pemphigus | Research article

Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies

Authors: Farnam Mohebi, Soheil Tavakolpour, Amir Teimourpour, Roja Toosi, Hamidreza Mahmoudi, Kamran Balighi, Narges Ghandi, Maryam Ghiasi, Pedram Nourmohammadpour, Vahideh Lajevardi, Robabeh Abedini, Armaghan Azizpour, Maryam Nasimi, Maryam Daneshpazhooh

Published in: BMC Dermatology | Issue 1/2020

Login to get access

Abstract

Background

Pemphigus is a potentially fatal disease if left untreated. Valid scoring systems and defined cut-off values for classification of patients would help with better management through specified pharmaceutical and non-pharmaceutical treatments.

Methods

In this study, pemphigus patients who were receiving immunosuppressive treatments and had recent disease relapse were recruited for examination of pemphigus disease area index(PDAI), autoimmune bullous skin disorder intensity score (ABSIS), physician global assessment (PGA), autoimmune bullous disease quality of life (ABQoL), anti-desmoglein 1 (anti-Dsg1), and anti-Dsg3 autoantibody titers from December-2017 to February-2018. Cut-off values were estimated using model-based clustering classification and the 25th and 75th percentiles approach, performed separately for the exclusive cutaneous, exclusive mucosal, and mucocutaneous groups.

Results

In the 109 included patients, the 25th and 75th percentiles cut-offs were 6.2 and 27 for PDAI score, and 4 and 29.5 for ABSIS score. The model-based analysis resulted in two groups (cut-point:15) for PDAI score, and three groups (cut-points:6.4 and 31.5) for ABSIS score. The groups were significantly different for the PDAI, ABSIS, PGA, and ABQoL values. Based on anti-Dsg1 autoantibody values, the model-based analysis cut-point was 128 and the 25th and 75th percentiles cut-offs were 98 and 182. Anti-Dsg3 autoantibody values did not differentiate between pemphigus severity classes.

Conclusions

Estimated cut-off values based on the anti-Dsg1 level, PDAI, and ABSIS scoring systems could be used to classify patients into different severity grades for better management and prognosis.
Literature
2.
go back to reference Joly P, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020, 1900;34(9):–13. Joly P, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020, 1900;34(9):–13.
3.
go back to reference Pascoe VL, et al. Using the physician global assessment in a clinical setting to measure and track patient outcomes. JAMA Dermatol. 2015;151(4):375–81.CrossRefPubMed Pascoe VL, et al. Using the physician global assessment in a clinical setting to measure and track patient outcomes. JAMA Dermatol. 2015;151(4):375–81.CrossRefPubMed
4.
go back to reference Hanna S, Kim M, Murrell DF. Validation studies of outcome measures in pemphigus. Int J Women’s Dermatol. 2016;2(4):128–39.CrossRef Hanna S, Kim M, Murrell DF. Validation studies of outcome measures in pemphigus. Int J Women’s Dermatol. 2016;2(4):128–39.CrossRef
6.
go back to reference Pfutze M, et al. Introducing a novel autoimmune bullous skin disorder intensity score (ABSIS) in pemphigus. Eur J Dermatol. 2007;17(1):4–11.PubMed Pfutze M, et al. Introducing a novel autoimmune bullous skin disorder intensity score (ABSIS) in pemphigus. Eur J Dermatol. 2007;17(1):4–11.PubMed
7.
go back to reference Zhao CY, Murrell DF. Outcome measures for autoimmune blistering diseases. J Dermatol. 2015;42(1):31–6.CrossRefPubMed Zhao CY, Murrell DF. Outcome measures for autoimmune blistering diseases. J Dermatol. 2015;42(1):31–6.CrossRefPubMed
8.
go back to reference Hebert V, et al. Large international validation of ABSIS and PDAI pemphigus severity scores. J Invest Dermatol. 2019;139(1):31–7.CrossRefPubMed Hebert V, et al. Large international validation of ABSIS and PDAI pemphigus severity scores. J Invest Dermatol. 2019;139(1):31–7.CrossRefPubMed
9.
go back to reference Murrell DF, et al. Diagnosis and Management of Pemphigus: recommendations by an international panel of experts. J Am Acad Dermatol. 2018. Murrell DF, et al. Diagnosis and Management of Pemphigus: recommendations by an international panel of experts. J Am Acad Dermatol. 2018.
10.
go back to reference Kalinska-Bienias A, et al. Measuring of quality of life in autoimmune blistering disorders in Poland. Validation of disease – specific autoimmune bullous disease quality of life (ABQOL) and the treatment autoimmune bullous disease quality of life (TABQOL) questionnaires. Adv Med Sci. 2017;62(1):92–6.CrossRefPubMed Kalinska-Bienias A, et al. Measuring of quality of life in autoimmune blistering disorders in Poland. Validation of disease – specific autoimmune bullous disease quality of life (ABQOL) and the treatment autoimmune bullous disease quality of life (TABQOL) questionnaires. Adv Med Sci. 2017;62(1):92–6.CrossRefPubMed
11.
go back to reference Teimourpour A, et al. Autoimmune Bullous Disease Quality of Life (ABQOL) Questionnaire: Validation of the translated Persian version in pemphigus vulgaris. Int J Womens Dermatology. 2020. Teimourpour A, et al. Autoimmune Bullous Disease Quality of Life (ABQOL) Questionnaire: Validation of the translated Persian version in pemphigus vulgaris. Int J Womens Dermatology. 2020.
12.
go back to reference Fraley C, Raftery AE. MCLUST version 3: an R package for normal mixture modeling and model-based clustering. Washington: University Seattle Department of Statistics; 2006.CrossRef Fraley C, Raftery AE. MCLUST version 3: an R package for normal mixture modeling and model-based clustering. Washington: University Seattle Department of Statistics; 2006.CrossRef
13.
go back to reference Boulard C, et al. 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. Br J Dermatol. 2016;175(1):142–9.CrossRefPubMed Boulard C, et al. 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. Br J Dermatol. 2016;175(1):142–9.CrossRefPubMed
14.
go back to reference Shimizu T, et al. Grading criteria for disease severity by pemphigus disease area index. J Dermatol. 2014;41(11):969–73.CrossRefPubMed Shimizu T, et al. Grading criteria for disease severity by pemphigus disease area index. J Dermatol. 2014;41(11):969–73.CrossRefPubMed
15.
go back to reference Abasq C, et al. ELISA testing of anti–desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 2009;145(5):529–35.CrossRefPubMed Abasq C, et al. ELISA testing of anti–desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 2009;145(5):529–35.CrossRefPubMed
16.
go back to reference Albers LN, et al. Developing biomarkers for predicting clinical relapse in pemphigus patients treated with rituximab. J Am Acad Dermatol. 2017;77(6):1074–82.CrossRefPubMed Albers LN, et al. Developing biomarkers for predicting clinical relapse in pemphigus patients treated with rituximab. J Am Acad Dermatol. 2017;77(6):1074–82.CrossRefPubMed
17.
go back to reference Didona D, et al. Pemphigus: current and future therapeutic strategies. Front Immunol. 2019;10. Didona D, et al. Pemphigus: current and future therapeutic strategies. Front Immunol. 2019;10.
18.
go back to reference Daneshpazhooh M, et al. Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission. J Am Acad Dermatol. 2016;74(6):1160–5.CrossRefPubMed Daneshpazhooh M, et al. Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission. J Am Acad Dermatol. 2016;74(6):1160–5.CrossRefPubMed
19.
go back to reference Mobarakabadi M, et al. Evaluation of the possible association between acantholysis and anti-desmogleins 1 and 3 values in pemphigus vulgaris and pemphigus foliaceus. J Cutan Immunol Allergy. 2019;2(6):169–73.CrossRef Mobarakabadi M, et al. Evaluation of the possible association between acantholysis and anti-desmogleins 1 and 3 values in pemphigus vulgaris and pemphigus foliaceus. J Cutan Immunol Allergy. 2019;2(6):169–73.CrossRef
20.
go back to reference Balighi K, et al. Anti-desmoglein-1 levels as predictor of prednisolone tapering in pemphigus vulgaris patients treated with rituximab. Dermatol Ther. 2018;31(6):e12671.CrossRefPubMed Balighi K, et al. Anti-desmoglein-1 levels as predictor of prednisolone tapering in pemphigus vulgaris patients treated with rituximab. Dermatol Ther. 2018;31(6):e12671.CrossRefPubMed
21.
go back to reference Daneshpazhooh M, et al. Comparison of desmoglein 1 and 3 enzyme-linked immunosorbent assay and direct immunofluorescence for evaluation of immunological remission in pemphigus vulgaris. Clin Exp Dermatol. 2014;39(1):41–7.CrossRefPubMed Daneshpazhooh M, et al. Comparison of desmoglein 1 and 3 enzyme-linked immunosorbent assay and direct immunofluorescence for evaluation of immunological remission in pemphigus vulgaris. Clin Exp Dermatol. 2014;39(1):41–7.CrossRefPubMed
22.
go back to reference Tsunoda K, et al. Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3. J Immunol. 2003;170(4):2170–8.CrossRefPubMed Tsunoda K, et al. Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3. J Immunol. 2003;170(4):2170–8.CrossRefPubMed
23.
go back to reference Kamiya K, et al. A higher correlation of the antibody activities against the calcium-dependent epitopes of desmoglein 3 quantified by ethylenediaminetetraacetic acid-treated enzyme-linked immunosorbent assay with clinical disease activities of pemphigus vulgaris. J Dermatol Sci. 2013;70(3):190–5.CrossRefPubMed Kamiya K, et al. A higher correlation of the antibody activities against the calcium-dependent epitopes of desmoglein 3 quantified by ethylenediaminetetraacetic acid-treated enzyme-linked immunosorbent assay with clinical disease activities of pemphigus vulgaris. J Dermatol Sci. 2013;70(3):190–5.CrossRefPubMed
24.
go back to reference Daneshpazhooh M, et al. Comparison of ethylenediaminetetraacetic acid-treated desmoglein ELISA and conventional desmoglein ELISA in the evaluation of pemphigus vulgaris in remission. J Am Acad Dermatol. 2018;79(4):768–70.CrossRefPubMed Daneshpazhooh M, et al. Comparison of ethylenediaminetetraacetic acid-treated desmoglein ELISA and conventional desmoglein ELISA in the evaluation of pemphigus vulgaris in remission. J Am Acad Dermatol. 2018;79(4):768–70.CrossRefPubMed
25.
go back to reference Alaeen H, et al. Short-term clinical and serological follow-up with conventional and conformational anti-desmoglein antibodies in treatment-naïve and previously treated patients with pemphigus vulgaris after receiving rituximab. Int J Womens Dermatol. 2019;5(5):372–7.CrossRefPubMedPubMedCentral Alaeen H, et al. Short-term clinical and serological follow-up with conventional and conformational anti-desmoglein antibodies in treatment-naïve and previously treated patients with pemphigus vulgaris after receiving rituximab. Int J Womens Dermatol. 2019;5(5):372–7.CrossRefPubMedPubMedCentral
26.
go back to reference Dmochowski M, Gornowicz-Porowska J, Bowszyc-Dmochowska M. Dew drops on spider web appearance: a newly named pattern of IgG4 deposition in pemphigus with direct immunofluorescence. Adv Dermatol Allergol Postȩpy Dermatol Alergol. 2017;34(4):295.CrossRef Dmochowski M, Gornowicz-Porowska J, Bowszyc-Dmochowska M. Dew drops on spider web appearance: a newly named pattern of IgG4 deposition in pemphigus with direct immunofluorescence. Adv Dermatol Allergol Postȩpy Dermatol Alergol. 2017;34(4):295.CrossRef
28.
go back to reference Futei Y, et al. Predominant IgG4 subclass in autoantibodies of pemphigus vulgaris and foliaceus. J Dermatol Sci. 2001;26(1):55–61.CrossRefPubMed Futei Y, et al. Predominant IgG4 subclass in autoantibodies of pemphigus vulgaris and foliaceus. J Dermatol Sci. 2001;26(1):55–61.CrossRefPubMed
Metadata
Title
Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies
Authors
Farnam Mohebi
Soheil Tavakolpour
Amir Teimourpour
Roja Toosi
Hamidreza Mahmoudi
Kamran Balighi
Narges Ghandi
Maryam Ghiasi
Pedram Nourmohammadpour
Vahideh Lajevardi
Robabeh Abedini
Armaghan Azizpour
Maryam Nasimi
Maryam Daneshpazhooh
Publication date
01-12-2020
Publisher
BioMed Central
Published in
BMC Dermatology / Issue 1/2020
Electronic ISSN: 1471-5945
DOI
https://doi.org/10.1186/s12895-020-00105-y

Other articles of this Issue 1/2020

BMC Dermatology 1/2020 Go to the issue