Evidence map›Paper›PMID 34245180›Full record

GuidelineJournal of the European Academy of Dermatology and Venereology : JEADV2021

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

H Rashid, A Lamberts, L Borradori, S Alberti-Violetti, R J Barry, M Caproni, B Carey, M Carrozzo, F Caux, G Cianchini and 25 more

Open access · bronzeAbstract readPractice Guideline
In one paragraph

Guideline in Journal of the European Academy of Dermatology and Venereology : JEADV, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers, 5 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
59citing papers in PubMed, 5 pooled it
16.9field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

59 citing papers in PubMed, 5 syntheses or guidelines pooled it, 149 citations in OpenAlex.

  1. European S2k guidelines on management of autoimmune blistering diseases in children and adolescents.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026
    Guideline
  2. Pooled it
  3. Guideline
  4. Pooled it
  5. Pooled it
  6. [New treatment options for autoimmune bullous diseases].Dermatologie (Heidelberg, Germany) · 2026
    Review
  7. Tocilizumab for severe and refractory mucous membrane pemphigoid.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026
    Article
  8. Article
  9. Article
  10. Review
  11. Review
  12. Article
  13. Article
  14. Refractory ocular mucous membrane pemphigoid treated successfully with upadacitinib.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2026
    Article
  15. Article
  16. Characterization of mucous membrane pemphigoid in childhood: A multicentre study of 12 cases.Journal of the European Academy of Dermatology and Venereology : JEADV · 2025
    Article
  17. Article
  18. Article
  19. Review
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

35 authors at 20 institutions in 8 countries.

H RashidDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0002-9725-5207
A LambertsDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0001-6357-5264
L BorradoriDepartment of Dermatology, Inselspital, Bern University Hospital, Bern, Switzerland.ORCID https://orcid.org/0000-0003-0424-6297
S Alberti-ViolettiDermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
R J BarryAcademic Unit of Ophthalmology, Birmingham & Midland Eye Centre, University of Birmingham, Birmingham, UK.ORCID https://orcid.org/0000-0002-6435-0048
M CaproniDepartment of Health Sciences, Section of Dermatology, AUSL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence, Italy.ORCID https://orcid.org/0000-0002-7471-3471
B CareyDepartment of Oral Medicine, Guy's & St Thomas' NHS Foundation Trust, London, UK.ORCID https://orcid.org/0000-0002-6376-9867
M CarrozzoDepartment of Oral Medicine, School of Dental Sciences, Newcastle University, Newcastle, UK.
F CauxDepartment of Dermatology and Referral center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP and University Paris 13, Bobigny, France.ORCID https://orcid.org/0000-0001-9552-0882
G CianchiniDepartment of Dermatology, Ospedale Classificato Cristo Re, Rome, Italy.
A CorràDepartment of Health Sciences, Section of Dermatology, AUSL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence, Italy.ORCID https://orcid.org/0000-0003-0903-4998
G F H DiercksDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0001-8053-216X
F G DikkersDepartment of Otorhinolaryngology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.ORCID https://orcid.org/0000-0001-5265-3722
G Di ZenzoLaboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy.
C FelicianiDermatology unit, Department of Medicine and Surgery, University Hospital, University of Parma, Parma, Italy.ORCID https://orcid.org/0000-0003-0883-3210
G GeerlingDepartment of Ophthalmology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.ORCID https://orcid.org/0000-0003-2034-3687
G GenoveseDermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID https://orcid.org/0000-0002-7636-958X
M HertlDepartment of Dermatology and Allergology, Philipps-Universität, Marburg, Germany.
P JolyDepartment of Dermatology, Rouen University Hospital, Centre de Référence des Maladies Bulleuses Autoimmunes, and INSERM U1234, Normandie University, Rouen, France.ORCID https://orcid.org/0000-0002-5734-0255
A V MarzanoDermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID https://orcid.org/0000-0002-8160-4169
J M MeijerDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0001-7654-3528
V MercadanteDepartment of Oral Medicine, Eastman Dental Institute, University College London, London, UK.
D F MurrellDepartment of Dermatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia.
M OrmondDepartment of Oral Medicine, Guy's & St Thomas' NHS Foundation Trust, London, UK.ORCID https://orcid.org/0000-0002-2586-2200
H H PasDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
A Patsatsi2nd Department of Dermatology, Autoimmune Bullous Diseases Unit, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece.ORCID https://orcid.org/0000-0001-9616-2001
C ProstDepartment of Dermatology and Referral center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP and University Paris 13, Bobigny, France.
S RauzAcademic Unit of Ophthalmology, Birmingham & Midland Eye Centre, University of Birmingham, Birmingham, UK.ORCID https://orcid.org/0000-0003-4627-3496
B D van RhijnDepartment of Dermatology & Allergology, University Medical Center Utrecht, Utrecht, The Netherlands.
M RothDepartment of Ophthalmology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
E SchmidtDepartment of Dermatology and Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.ORCID https://orcid.org/0000-0002-1206-8913
J SetterfieldDepartment of Oral Medicine, Guy's & St Thomas' NHS Foundation Trust, London, UK.
G ZambrunoGenodermatosis Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.ORCID https://orcid.org/0000-0002-1295-056X
D ZillikensDepartment of Dermatology and Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.ORCID https://orcid.org/0000-0002-5668-307X
B HorváthDepartment of Dermatology, Center for Blistering Diseases, European Reference Network-Skin Member, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0001-8559-3674
University Medical Center Groningen · NLUniversity of Milan · ITBirmingham and Midland Eye Centre · GBDüsseldorf University Hospital · DEGuy's and St Thomas' NHS Foundation Trust · GBUniversité Sorbonne Paris Nord · FRUniversity of Florence · ITUniversity of Lübeck · DEAmsterdam University Medical Centers · NLBambino Gesù Children's Hospital · ITEastman Dental Hospital · GBInserm · FRIstituto Dermopatico dell'Immacolata · ITKing's College London · GBNewcastle University · GBOspedale Cristo Re · ITPapageorgiou General Hospital · GRPhilipps University of Marburg · DEUniversity Hospital of Bern · CHUniversity Medical Center Utrecht · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

DermatologyPemphigoid, Benign Mucous MembranePemphigoid, BullousVenereologyAutoantibodiesAutoantigensHumansMucous MembraneQuality of LifeSystematic Reviews as TopicAutoantibodiesAutoantigens

Identifiers

PMID34245180
PMCPMC8457055
OpenAlexW3181848860

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.