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 II.
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 77 papers, 5 of them syntheses that pooled it.
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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.
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Who cites it
77 citing papers in PubMed, 5 syntheses or guidelines pooled it.
- European S2k guidelines on management of autoimmune blistering diseases in children and adolescents.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026Guideline
- The efficacy and safety of pharmacological treatment of desquamative gingivitis: a systematic review.BMC oral health · 2025Pooled it
- Survey: Preferred practice patterns in the diagnosis of mucous membrane pemphigoid amongst cornea specialists.European journal of ophthalmology · 2024Guideline
- S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).Journal of the European Academy of Dermatology and Venereology : JEADV · 2023Guideline
- Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients.Frontiers in immunology · 2022Pooled it
- Prospective management of biopsy-proven ocular mucous membrane pemphigoid using an inflammation-oriented stepladder treatment strategy.AJO international · 2026Article
- Tocilizumab for severe and refractory mucous membrane pemphigoid.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026Article
- [New treatment options for autoimmune bullous diseases].Dermatologie (Heidelberg, Germany) · 2026Review
- Rituximab in Mucous Membrane Pemphigoid: Current Evidence and Clinical Perspectives.Healthcare (Basel, Switzerland) · 2026Article
- Conjunctival biopsy findings associated with fibrosis and sequelae in ocular Mucous membrane pemphigoid.International ophthalmology · 2026Article
- Mucous Membrane Pemphigoid during CPX-351 Therapy Successfully Treated with Corticosteroids.Internal medicine (Tokyo, Japan) · 2026Article
- Autoimmune Oral Mucosal Diseases in Older Patients: An Overview of Early Recognition and Management in the Primary Care Setting.Geriatrics (Basel, Switzerland) · 2026Review
- Immunochemical Determination of IgE and IgG Autoantibodies in Patients with Chronic Spontaneous Urticaria: A Narrative Review.Antibodies (Basel, Switzerland) · 2026Review
- Autoimmune Bullous Diseases: Therapeutic Update.Drugs · 2026Review
- Complement Activation May Drive the Pathogenicity of Anti-α6 and Anti-β4 Integrin Antibodies In Vivo.Biomolecules · 2026Article
- Flow cytometry helps differentiate between mucous membrane pemphigoid and erosive oral lichen planus.Scientific reports · 2026Article
- Dupilumab enables glucocorticoid withdrawal in refractory multisite mucous membrane pemphigoid: a case report.Frontiers in medicine · 2026Article
- Refractory ocular mucous membrane pemphigoid treated successfully with upadacitinib.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2026Article
- Characterization of mucous membrane pemphigoid in childhood: A multicentre study of 12 cases.Journal of the European Academy of Dermatology and Venereology : JEADV · 2025Article
- Mucous Membrane Pemphigoid Outcome Measurement in Clinical Research: A Scoping Review.Clinical drug investigation · 2025Article
17 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
35 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue-bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10-25% of patients laminin 332 is recognized. In 25-30% of MMP patients with anti-laminin 332 reactivity, malignancies have been associated. As first-line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first-line regimens. Additional recommendations are given, tailored to treatment of single-site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high-quality randomized controlled trials.
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