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1. Actualisation des recommandations françaises de traitement du pemphigus

Author

Jelti, L., Prost-Squarcioni, C., Ingen-Housz-Oro, S., Caux, F., Bernard, P., Bedane, C., Alexandre, M., Dereure, O., Quereux, G., Le Bidre, E., Plée, J., Picard-Dahan, C., Le Roux-Villet, C., Duvert-Lehembre, S., Richard, M.-A., Delaporte, E., Debarbieux, S., Jullien, D., D’Incan, M., Konstantinou, M.-P., Bouaziz, J.-D., Tancrède-Bohin, E., Doutre, M.-S., Bourgault Villada, I., Cordel, N., Sassolas, B., Viguier, M.-A., Mellottée, B., Jouen, F., Hebert, V., and Joly, P.

Published
2019
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7. Efficacité et tolérance à long terme des schémas thérapeutiques testés chez les patients atteints de pemphigus inclus dans l’essai clinique Ritux 3

Author

Tedbirt, B., primary, Maho-Vaillant, M., additional, Houivet, E., additional, Calbo, S., additional, Prost-Squarcioni, C., additional, Labeille, B., additional, Picard-Dahan, C., additional, Konstantinou, M.P., additional, Chaby, G., additional, Richard, M.A., additional, Bouaziz, J.D., additional, Duvert Lehembre, S., additional, Delaporte, E., additional, Bernard, P., additional, Caux, F., additional, Alexandre, M., additional, Oro, S., additional, Vabres, P., additional, Quéreux, G., additional, Dupuy, A., additional, Debarbieux, S., additional, Avenel-Audran, M., additional, D’Incan, M., additional, Bedane, C., additional, Beneton, N., additional, Jullien, D., additional, Dupin, N., additional, Misery, L., additional, Machet, L., additional, Beylot-Barry, M., additional, Dereure, O., additional, Sassolas, B., additional, Benichou, J., additional, Joly, P., additional, and Hébert, V., additional

Published
2022
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11. Épidermolyse bulleuse acquise de l’enfant : série de 17 cas

Author

Tzoumpa, S., Bohelay, G., Alexandre, M., Sitbon, I., Soued, I., Welfringer-Morin, A., Grootenboer-Mignot, S., Hovnanian, A., Caux, F., Prost-Squarcioni, C., and Le Roux-Villet, C.

Abstract

L’épidermolyse bulleuse acquise (EBA) est une maladie bulleuse auto-immune secondaire à la production d’auto-anticorps (ac) dirigés contre le collagène VII. Chez l’enfant, cette maladie est exceptionnelle, avec 73 cas rapportés à ce jour. Nous rapportons ici une série rétrospective de 17 cas pédiatriques d’EBA.

Published
2024
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18. Comparaison de l’évolution de la qualité de vie sous traitement des patients atteints de pemphigoïde bulleuse, pemphigoïde cicatricielle ou pemphigus

Author

Huard, E., primary, Gillibert, A., additional, Ferries, L., additional, Alexandre, M., additional, Duvert-Lehembre, S., additional, Corbaux, C., additional, Prost-Squarcioni, C., additional, Caux, F., additional, Delaporte, E., additional, Litrowski, N., additional, Boulard, C., additional, Joly, P., additional, and Hebert, V., additional

Published
2020
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20. Incidence et pronostic de la COVID-19 chez les malades atteints de maladie bulleuse auto-immune

Author

Aouar, K., primary, Guillibert, A., additional, Bohelay, G., additional, Le Roux-Villet, C., additional, Alexandre, M., additional, Prost-Squarcioni, C., additional, Musette, P., additional, Tancrede Bohin, E., additional, Viguier, M., additional, Dereure, O., additional, Picard Dahan, C., additional, Bedane, C., additional, Morice, C., additional, Debarbieux, S., additional, Jullien, D., additional, Fenot, M., additional, d’Incan, M., additional, Aubin, F., additional, Cordel, N., additional, Mahe, A., additional, Perrot, J.-L., additional, Pham-Ledard, A., additional, Jeudy, G., additional, Oro, S., additional, Lepelletier, C., additional, Dupin, N., additional, Seta, V., additional, Finon, A., additional, Lehembre, S. Duvert, additional, Mahe, E., additional, Cribier, B., additional, Schmutz, J.-L., additional, Templier, I., additional, Chaby, G., additional, Abasq, C., additional, Quereux Baumgartner, G., additional, Maillard, H., additional, Kupfer, I., additional, Richard, M.-A., additional, Le Duff, F., additional, Konstantinou, M.P., additional, Litrowski, N., additional, Castel, M., additional, Ramstein, M., additional, Muller, P., additional, Le Bidre, E., additional, Caux, F., additional, and Joly, P., additional

Published
2020
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21. Sensitivity to change and correlation between the autoimmune bullous disease quality‐of‐life questionnaires ABQOL and TABQOL , and objective severity scores

Author

Ferries, L., primary, Gillibert, A., additional, Duvert‐Lehembre, S., additional, Corbaux, C., additional, Alexandre, M., additional, Prost‐Squarcioni, C., additional, Caux, F., additional, Delaporte, E., additional, Litrowski, N., additional, Boulard, C., additional, Murrell, D.F., additional, Joly, P., additional, and Hébert, V., additional

Published
2020
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26. Comparaison de l’efficacité et de la tolérance à long terme du rituximab entre les patients atteints de pemphigus foliacé et de pemphigus vulgaire

Author

Tedbirt, B., Houivet, E., Maho-Vaillant, M., Golinski, M.L., Calbo, S., Prost-Squarcioni, C., Labeille, B., Picard-Dahan, C., Chaby, G., Richard, M.A., Tancrede, E., Duvert Lehembre, S., Delaporte, E., Bernard, P., Caux, F., Alexandre, M., Musette, P., Oro, S., Vabres, P., Quéreux, G., Dupuy, A., Debarbieux, S., Avenel-Audran, M., D’incan, M., Bedane, C., Beneton, N., Jullien, D., Dupin, N., Misery, L., Machet, L., Beylot-Barry, M., Dereure, O., Sassolas, B., Cordel, N., Jeudy, G., Benichou, J., Hebert, V., and Joly, P.

Abstract

L’essai Ritux 3 a permis l’obtention de l’AMM du rituximab pour le traitement du pemphigus vulgaire (PV) modéré à sévère. En raison du nombre limité de patients atteints de pemphigus foliacé (PF) ou superficiel inclus dans l’essai, cette autorisation n’a pas été étendue aux PF. Il est suggéré dans la littérature que l’efficacité du rituximab serait moindre dans les PF que dans les PV. L’objectif de notre étude était de comparer l’efficacité et la tolérance à long terme du rituximab chez les patients atteints de PF par rapport à ceux atteints de PV.

Published
2024
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27. Efficacité et tolérance de la prednisone à 0,5 mg/kg/j en traitement initial de la pemphigoïde bulleuse

Author

Bastos, S., primary, Hebert, V., additional, Vassileva, S., additional, Patsatsi, A., additional, Meijer, J., additional, Quéreux, G., additional, Bedane, C., additional, Prost-Squarcioni, C., additional, Debardieux, S., additional, Oro, S., additional, Chaby, G., additional, D’Incan, M., additional, Litrowski, N., additional, Boulard, C., additional, Lunardon, L., additional, Kiritsi, D., additional, Jedlickova, H., additional, Feliciani, C., additional, Roussel, A., additional, Kottler, D., additional, Plantin, P., additional, Richard, M.-A., additional, Friedrichsen, L., additional, Abasq, C., additional, Duvert Lehembre, S., additional, Gottlieb, J., additional, Zebrowska, A., additional, Hofmann, S., additional, and Joly, P., additional

Published
2019
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28. Évaluation d’un questionnaire de sévérité au cours du pemphigus

Author

Pinard, C., primary, Duvert-Lehembre, S., additional, Prost-Squarcioni, C., additional, Alsubaiei, F., additional, Houivet, E., additional, Le Henaff, Y., additional, Heas, S., additional, Picard-Dahan, C., additional, Bernard, P., additional, Doutre, M.S., additional, Beylot-Barry, M., additional, Richard, M.-A., additional, Delaporte, E., additional, Fleuret, C., additional, Oro, S., additional, D’Incan, M., additional, Alexandre, M., additional, Caux, F., additional, Maillard, H., additional, Bénéton, N., additional, Hebert, V., additional, and Joly, P., additional

Published
2019
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29. Corrélations entre les questionnaires de qualité de vie spécifiques aux maladies bulleuses auto-immunes ABQOL et TABQOL, et les scores de sévérité objectifs

Author

Ferries, L., primary, Gilibert, A., additional, Alexandre, M., additional, Duvert-Lehembre, S., additional, Corbaux, C., additional, Hébert, V., additional, Prost-Squarcioni, C., additional, Caux, F., additional, Delaporte, E., additional, Litrowski, N., additional, Boulard, C., additional, Murrell, D., additional, and Joly, P., additional

Published
2019
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30. Facteurs pronostiques de rechute à long terme chez les patients atteints de pemphigus traités par rituximab en première ligne

Author

Mignard, C., primary, Maho-Vaillant, M., additional, Prost-Squarcioni, C., additional, Calbo, S., additional, Golinski, M.-L., additional, Labeille, B., additional, Picard-Dahan, C., additional, Konstantinou, M.P., additional, Richard, M.-A., additional, Bouaziz, J.D., additional, Duvert-Lehembre, S., additional, Bernard, P., additional, Caux, F., additional, Alexandre, M., additional, Oro, S., additional, Vabres, P., additional, Quereux, G., additional, Dupuy, A., additional, Debarbieux, S., additional, Martin, L., additional, D’Incan, M., additional, Bedane, C., additional, Bénéton, N., additional, Jullien, D., additional, Dupin, N., additional, Misery, L., additional, Machet, L., additional, Beylot-Barry, M., additional, Dereure, O., additional, Sassolas, B., additional, Hébert, V., additional, and Joly, P., additional

Published
2019
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32. 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.

Author

Masmoudi, W., Vaillant, M., Vassileva, S., Patsatsi, A., Quereux, G., Moltrasio, C., Abasq, C., Prost‐Squarcioni, C., Kottler, D., Kiritsi, D., Litrowski, N., Plantin, P., Friedrichsen, L., Zebrowska, A., Duvert‐Lehembre, S., Hofmann, S., Ferranti, V., Jouen, F., Joly, P., and Hebert, V.

Subjects
BULLOUS pemphigoid, PHYSICIANS, ENZYME-linked immunosorbent assay, INTRACLASS correlation, DIAGNOSIS, INTER-observer reliability
Abstract

Summary: 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. What is already known about this topic?The Bullous Pemphigoid Disease Area Index (BPDAI) is a new scoring system to measure bullous pemphigoid (BP) activity.The use of this score in clinical practice is limited by the absence of cut‐off values. What does this study add?Cut‐off values of 20 and 57 were established to distinguish mild, moderate and severe BP using the BPDAI.These disease activity subgroups could help physicians in the management of patients with BP. What are the clinical implications of this work?This study classified patients with BP into three subgroups (mild, moderate and severe) based on the BPDAI score.The BPDAI score is a robust tool to assess BP activity accurately, both at diagnosis and during the course of the disease, and could help physicians tailor treatment to disease activity, thereby providing better management of patients with BP. Linked Comment: Blome and Klein. Br J Dermatol 2021; 184:997–998. [ABSTRACT FROM AUTHOR]

Published
2021
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33. International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita

Author

Prost-Squarcioni, C., Caux, F., Schmidt, E., Jonkman, M.F., Vassileva, S., Kim, S.C., Iranzo, P., Daneshpazhooh, M., Terra, J., Bauer, J., Fairley, J., Hall, R., Hertl, M., Lehman, J.S., Marinovic, Branka, Patsatsi, A., Zillikens, D., Werth, V., Woodley, D.T., Murrell, D.F., and the International Bullous Diseases Group

Subjects
Epidermolysis bullosa acquisita, SERRATION PATTERN-ANALYSIS, Microscopy, Electron, Scanning Transmission, medicine.medical_specialty, epidermolysis bullosa acquisita, diagnostic criteria, consensus conference, Consensus, media_common.quotation_subject, education, Immunoblotting, MEDLINE, Fluorescent Antibody Technique, Enzyme-Linked Immunosorbent Assay, Dermatology, Disease, Review, Epidermolysis Bullosa Acquisita, VII COLLAGEN ANTIBODIES, Diagnosis, Differential, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Voting, medicine, Journal Article, Bullous disease, Humans, Intensive care medicine, Microscopy, Immunoelectron, health care economics and organizations, media_common, SPLIT-SKIN, LINKED-IMMUNOSORBENT-ASSAY, DIRECT IMMUNOFLUORESCENCE MICROSCOPY, business.industry, Clinical Laboratory Techniques, Consensus conference, OUTCOME MEASURES, medicine.disease, SCANNING CONFOCAL MICROSCOPY, CROHNS-DISEASE, Flow chart, 030220 oncology & carcinogenesis, Differential diagnosis, business, CHLORIDE-SEPARATED SKIN, LINEAR IGA DISEASE
Abstract

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential. ----- OBJECTIVES: To obtain an international consensus on the clinical and diagnostic criteria for EBA. ----- METHODS: The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out. ----- RESULTS: In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed. ----- CONCLUSIONS: This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting.

Published
2017

34. Rituximab is an effective treatment in patients with pemphigus vulgaris and demonstrates a steroid‐sparing effect.

Author

Chen, D.M., Odueyungbo, A., Csinady, E., Gearhart, L., Lehane, P., Cheu, M., Maho‐Vaillant, M., Prost‐Squarcioni, C., Hebert, V., Houivet, E., Calbo, S., Caillot, F., Golinski, M.L., Labeille, B., Picard‐Dahan, C., Paul, C., Richard, M.A., Bouaziz, J.D., Duvert‐Lehembre, S., and Bernard, P.

Subjects
RITUXIMAB, IMMUNOSUPPRESSIVE agents, REGULATORY approval, PEMPHIGUS, PREDNISONE
Abstract

Summary: Background: Corticosteroids (CS) with or without adjuvant immunosuppressant agents are standard treatment for pemphigus vulgaris (PV). The efficacy of adjuvant therapies in minimizing steroid‐related adverse events (AEs) is unproven. Objectives: To utilize data collected in a French investigator‐initiated, phase III, open‐label, randomized controlled trial to demonstrate the efficacy and safety of rituximab and seek approval for its use in PV. Methods: This was an independently conducted post hoc analysis of the moderate‐to‐severe PV subset enrolled in the Ritux 3 study. Patients were randomized to rituximab plus 0·5 or 1·0 mg kg−1 per day prednisone tapered over 3 or 6 months, or 1·0 or 1·5 mg kg−1 per day prednisone alone tapered over 12 or 18 months, respectively (according to disease severity). The primary end point was complete remission at month 24 without CS (CRoff) for ≥ 2 months, and 24‐month efficacy and safety results were also reported. Results: At month 24, 34 of 38 patients (90%) on rituximab plus prednisone achieved CRoff ≥ 2 months vs. 10 of 36 patients (28%) on prednisone alone. Median total cumulative prednisone dose was 5800 mg in the rituximab plus prednisone arm vs. 20 520 mg for prednisone alone. Eight of 36 patients (22%) who received prednisone alone withdrew from treatment owing to AEs; one rituximab‐plus‐prednisone patient withdrew due to pregnancy. Overall, 24 of 36 patients (67%) on prednisone alone experienced a grade 3/4 CS‐related AE vs. 13 of 38 patients (34%) on rituximab plus prednisone. Conclusions: In patients with moderate‐to‐severe PV, rituximab plus short‐term prednisone was more effective than prednisone alone. Patients treated with rituximab had less CS exposure and were less likely to experience severe or life‐threatening CS‐related AEs. What's already known about this topic? Pemphigus vulgaris (PV) is the most common type of pemphigus.Corticosteroids, a standard first‐line treatment for PV, have significant side‐effects.Although their effects are unproven, adjuvant corticosteroid‐sparing agents are routinely used to minimize steroid exposure and corticosteroid‐related side‐effects.There is evidence that the anti‐CD20 antibody rituximab is effective in the treatment of patients with severe recalcitrant pemphigus and in patients with newly diagnosed pemphigus. What does this study add? This study provides a more detailed analysis of patients with PV enrolled in an investigator‐initiated trial.Rituximab plus prednisone had a steroid‐sparing effect and more patients achieved complete remission off prednisone.Fewer patients experienced grade 3 or grade 4 steroid‐related adverse events than those on prednisone alone.This collaboration between academia and industry, utilizing independent post hoc analyses, led to regulatory authority approvals of rituximab in moderate‐to‐severe PV. Linked Comment: Scorer et al. Br J Dermatol 2020; 182:1078–1079. [ABSTRACT FROM AUTHOR]

Published
2020
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37. Comparaison des coûts de santé du traitement de première ligne par rituximab par rapport à la corticothérapie standard chez les patients pemphigus nouvellement diagnostiqués

Author

Hebert, V., primary, Vermeulin, T., additional, Prost-Squarcioni, C., additional, Picard-Dahan, C., additional, Duvert-Lehembre, S., additional, Bruno, L., additional, Marie-Aleth, R., additional, Bernard, P., additional, Dupuy, A., additional, Bouaziz, J.-D., additional, Oro, S., additional, Dalac-Rat, S., additional, Delaporte, E., additional, Avenel-Audran, M., additional, Sassolas, B., additional, Alexandre, M., additional, Caux, F., additional, D’Incan, M., additional, Bedane, C., additional, Quereux, G., additional, Machet, L., additional, Dereure, O., additional, Skowron, F., additional, Franck, N., additional, Beylot-Barry, M., additional, Doutre, M.-S., additional, Debarbieux, S., additional, Jullien, D., additional, Misery, L., additional, Musette, P., additional, Paul, C., additional, Vabres, P., additional, Bénéton, N., additional, Dupin, N., additional, Houivet, E., additional, and Joly, P., additional

Published
2018
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38. Dermatose à IgA linéaire induite : étude nationale de 69 cas

Author

Garel, B., primary, Oro, S., additional, Afriat, D., additional, Prost-Squarcioni, C., additional, Tétart, F., additional, Bensaid, B., additional, Bara Passot, C., additional, Beylot-Barry, M., additional, Descamps, V., additional, Duvert-Lehembre, S., additional, Grootenbœr-Mignot, S., additional, Jeudy, G., additional, Soria, A., additional, Valnet-Rabier, M.-B., additional, Barbaud, A., additional, Caux, F., additional, and Lebrun-Vignes, B., additional

Published
2018
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39. Calcul des valeurs seuils du score BPDAI (Bullous Pemphigoid Disease Area Index) définissant les formes légères, modérées et sévères de la pemphigoïde bulleuse

Author

Masmoudi, W., primary, Hebert, V., additional, Quéreux, G., additional, Bedane, C., additional, Prost-Squarcioni, C., additional, Debarbieux, S., additional, Oro, S., additional, Chaby, G., additional, D’incan, M., additional, Litrowski, N., additional, Boulard, C., additional, Roussel, A., additional, Kottler, D., additional, Abasq, C., additional, Richard, M.-A., additional, Duvert-Lehembre, S., additional, Gottlieb, J., additional, Plantin, P., additional, and Joly, P., additional

Published
2018
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41. 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

Author

Boulard, C., Duvert Lehembre, S., Picard-Dahan, C., Kern, J.S., Zambruno, G., Feliciani, C., Marinovic, B., Vabres, P., Borradori, L., Prost-Squarcioni, C., Labeille, B., Richard, M.A., Ingen-Housz-Oro, S., Houivet, E., Werth, V.P., Murrell, D.F., Hertl, M., Bénichou, J., Joly, P., Service de Dermatologie [Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Service de Dermatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de dermatologie [Avicenne], Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre de Recherches en Oncologie biologique et Oncopharmacologie (CRO2), Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie, vénéreologie et cancérologie cutanée [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de dermatologie [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Unité de biostatistiques [CHU Rouen], Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Pharmacoepidemiologie et évaluation de l'impact des produits de santé sur les populations, Université Bordeaux Segalen - Bordeaux 2-Université de Rouen Normandie (UNIROUEN), Hôpital Charles Nicolle [Rouen]-CHU Rouen, CHU Saint-Etienne, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Bordeaux Segalen - Bordeaux 2-Université de Rouen Normandie (UNIROUEN), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 (UPD7), Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Avicenne, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), and Unité de biostatistiques [Rouen]

Subjects
integumentary system, skin and connective tissue diseases, [SDV.MHEP.DERM]Life Sciences [q-bio]/Human health and pathology/Dermatology
Abstract

International audience; 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.

Published
2016
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42. Calculation of cut‐off values based on the Autoimmune Bullous Skin Disorder Intensity Score ( <scp>ABSIS</scp> ) and Pemphigus Disease Area Index ( <scp>PDAI</scp> ) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus

Author

Boulard, C., Duvert Lehembre, S., Picard‐Dahan, C., Kern, J.S., Zambruno, G., Feliciani, C., Marinovic, B., Vabres, P., Borradori, L., Prost‐Squarcioni, C., Labeille, B., Richard, M.A., Ingen‐Housz‐Oro, S., Houivet, E., Werth, V.P., Murrell, D.F., Hertl, M., Benichou, J., Joly, P., Hofmann, S., Camaioni, B., Didona, B., Fania, L., Della Torre, R., Caux, F., Alexandre, M., Paul, C., Bulai Livideanu, C., Delaporte, E., Dupuy, A., Avenel‐Audran, M., Schmidt, E., Schumacher, N., Bagot, M., Tancrede‐Bohin, E., Bouaziz, J.D., D'Incan, M., Quereux, G., Skowron, F., Beylot‐Barry, M., Doutre, M.S., Bedane, C., Bernard, P., Machet, L., Jullien, ., Debarbieux, S., Dereure, O., Hünefeld, C., Schanz, S., Franck, N., Maillard, H., Misery, L., Sassolas, B., Abasq, C., Bouyeure, A.C., Lagoutte, P., Ferranti, V., and the International Pemphigus Study Group

Subjects
pemphigus vulgaris, pemphigus foliaceus, severity scores
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.

Published
2016

46. Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults

Author

Gottlieb, J., primary, Ingen-Housz-Oro, S., additional, Alexandre, M., additional, Grootenboer-Mignot, S., additional, Aucouturier, F., additional, Sbidian, E., additional, Tancrede, E., additional, Schneider, P., additional, Regnier, E., additional, Picard-Dahan, C., additional, Begon, E., additional, Pauwels, C., additional, Cury, K., additional, Hüe, S., additional, Bernardeschi, C., additional, Ortonne, N., additional, Caux, F., additional, Wolkenstein, P., additional, Chosidow, O., additional, and Prost-Squarcioni, C., additional

Published
2017
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47. 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

Author

Boulard, C, Lehembre, SD, Picard-Dahan, C, Kern, JS, Zambruno, G, Feliciani, C, Marinovic, B, Vabres, P, Borradori, L, Prost-Squarcioni, C, Labeille, B, Richard, MA, Ingen-Housz-Oro, S, Houivet, E, Werth, VP, Murrell, DF, Hertl, M, Benichou, J, Joly, P, Boulard, C, Lehembre, SD, Picard-Dahan, C, Kern, JS, Zambruno, G, Feliciani, C, Marinovic, B, Vabres, P, Borradori, L, Prost-Squarcioni, C, Labeille, B, Richard, MA, Ingen-Housz-Oro, S, Houivet, E, Werth, VP, Murrell, DF, Hertl, M, Benichou, J, and Joly, P

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.

Published
2016

50. Morbidité et facteurs pronostiques dans la pemphigoïde cicatricielle : étude de cohorte rétrospective

Author

Seta, V., primary, Alexandre, M., additional, Zumelzu, C., additional, Oro, S., additional, Bernardeschi, C., additional, Schneider, P., additional, Le Roux-Villet, C., additional, Pascal, F., additional, Weber, P., additional, Doan, S., additional, Soued, I., additional, Raynaud, J.J., additional, Benamouzig, R., additional, Vibert, J.F., additional, Tancrède, E., additional, Dupin, N., additional, Laroche, L., additional, Caux, F., additional, Tubach, F., additional, Prost-Squarcioni, C., additional, and Gault, N., additional

Published
2016
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