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European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I

Research output: Contribution to journalArticlepeer-review

H. Rashid, A. Lamberts, L. Borradori, S. Alberti-Violetti, R. J. Barry, M. Caproni, B. Carey, M. Carrozzo, F. Caux, G. Cianchini, A. Corrà, G. F.H. Diercks, F. G. Dikkers, G. Di Zenzo, C. Feliciani, G. Geerling, G. Genovese, M. Hertl, P. Joly, A. V. Marzano & 15 more J. M. Meijer, V. Mercadante, D. F. Murrell, M. Ormond, H. H. Pas, A. Patsatsi, C. Prost, S. Rauz, B. D. van Rhijn, M. Roth, E. Schmidt, J. Setterfield, G. Zambruno, D. Zillikens, B. Horváth

Original languageEnglish
Pages (from-to)1750-1764
Number of pages15
JournalJournal of the European Academy of Dermatology and Venereology
Volume35
Issue number9
DOIs
Accepted/In press2021
PublishedSep 2021

Bibliographical note

Funding Information: M. Carrozzo received a grant from AFYX. F. Caux has been advisor, speaker or investigator for Principia Biopharma, Roche Laboratories, Pierre Fabre Dermatologie, LEO, Abbvie and Novartis. G. Geerling has been advisor, speaker or investigator for, and received grants from, Dompé, Chiesi, Novartis, Alcon, Allergan, Santen, Oculus, Tearlab, Tearscience, Theapharma and Visumed. B. Horvath has been advisor, speaker or investigator for, and received grants from, Abbvie, Janssen‐Cilag, Solenne B.V, Amgen, Akari Pharmaceutics, Roche, Novartis, UCB Pharma. P. Joly has been consultant for Roche, Amgen, Principia Biopharma, Argenx, AstraZeneca, Regeneron and Thermofisher. D.F. Murrell has been consultant, investigator, or speaker for Abbvie, Argenx, AstraZeneca, Dermira, Janssen, Lilly, Novartis, Principia Biopharma, Regeneron, Sanofi, UCB. A. Patsatsi has been advisor, speaker, or investigator for Abbvie, Jannsen‐Cilag, Lilly, Novartis, LEO, UCB, Principia Biopharma, L’Oreal and Genesis Pharma. M. Roth has been speaker for Theapharma and Bayer. E. Schmidt has been consultant for, and received grants and honoraria from, UCB, Biotest, Incyte, Euroimmun, Novartis, ArgenX, AstraZeneca, Fresenius Medical Care, Dompe, Synthon/byondis, Admirx, Topas, Thermo Fisher and Roche. G. Zambruno has been consultant for Argenx. D. Zillikens has been consultant, speaker, or investigator for Euroimmun, Almirall, UCB, Argenx, Biotest, Fresenius, Miltenyi, Roche, Biogen, Abbvie and Janssen. Publisher Copyright: © 2021 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology Copyright: Copyright 2021 Elsevier B.V., All rights reserved.

King's Authors

Abstract

This guideline on mucous membrane pemphigoid (MMP) has been elaborated by the Task Force for Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology (EADV) with a contribution of physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline encompassing a systematic review of the literature until June 2019 in the MEDLINE and EMBASE databases. This first part covers methodology, the clinical definition of MMP, epidemiology, MMP subtypes, immunopathological characteristics, disease assessment and outcome scores. MMP describes a group of autoimmune skin and mucous membrane blistering diseases, characterized by a chronic course and by predominant involvement of the mucous membranes, such as the oral, ocular, nasal, nasopharyngeal, anogenital, laryngeal and oesophageal mucosa. MMP patients may present with mono- or multisite involvement. Patients’ autoantibodies have been shown to be predominantly directed against BP180 (also called BPAG2, type XVII collagen), BP230, laminin 332 and type VII collagen, components of junctional adhesion complexes promoting epithelial stromal attachment in stratified epithelia. Various disease assessment scores are available, including the Mucous Membrane Pemphigoid Disease Area Index (MMPDAI), the Autoimmune Bullous Skin disorder Intensity Score (ABSIS), the ‘Cicatrising Conjunctivitis Assessment Tool’ and the Oral Disease Severity Score (ODSS). Patient-reported outcome measurements (PROMs), including DLQI, ABQOL and TABQOL, can be used for assessment of quality of life to evaluate the effectiveness of therapeutic interventions and monitor disease course.

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