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

被引：116

作者：

Schmidt, E. ^{[1
,2
]}

Rashid, H. ^{[3
]}

Marzano, A., V ^{[4
,5
]}

Lamberts, A. ^{[3
]}

Di Zenzo, G. ^{[6
]}

Diercks, G. F. H. ^{[3
,7
]}

Alberti-Violetti, S. ^{[4
,5
]}

Barry, R. J. ^{[8
]}

Borradori, L. ^{[9
]}

Caproni, M. ^{[10
]}

Carey, B. ^{[11
]}

Carrozzo, M. ^{[12
]}

Cianchini, G. ^{[13
]}

Corra, A. ^{[10
]}

Dikkers, F. G. ^{[14
]}

Feliciani, C. ^{[15
]}

Geerling, G. ^{[16
]}

Genovese, G. ^{[4
,5
]}

Hertl, M. ^{[17
]}

Joly, P. ^{[18
,19
]}

Meijer, J. M. ^{[3
]}

Mercadante, V ^{[20
]}

Murrell, D. F. ^{[21
]}

Ormond, M. ^{[11
]}

Pas, H. H. ^{[3
]}

Patsatsi, A. ^{[22
]}

Rauz, S. ^{[8
]}

van Rhijn, B. D. ^{[23
]}

Roth, M. ^{[16
]}

Setterfield, J. ^{[11
,24
,25
]}

Zillikens, D. ^{[1
]}

Prost, C. ^{[26
,27
,28
]}

Zambruno, G. ^{[29
]}

Horvath, B. ^{[3
]}

Caux, F. ^{[26
,27
,28
]}

机构：

[1] Univ Lubeck, Dept Dermatol, Lubeck, Germany

[2] Univ Lubeck, Lubeck Inst Expt Dermatol LIED, Lubeck, Germany

[3] Univ Groningen, Univ Med Ctr Groningen, Ctr Blistering Dis, Dept Dermatol, Groningen, Netherlands

[4] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dermatol Unit, Milan, Italy

[5] Univ Milan, Dept Pathophysiol & Transplantat, Milan, Italy

[6] IDI IRCCS, Ist Dermopat Immacolata, Lab Mol & Cell Biol, Rome, Italy

[7] Univ Groningen, Univ Med Ctr Groningen, Dept Pathol, Groningen, Netherlands

[8] Univ Birmingham, Birmingham & Midland Eye Ctr, Acad Unit Ophthalmol, Birmingham, W Midlands, England

[9] Bern Univ Hosp, Dept Dermatol, Inselspital, Bern, Switzerland

[10] Univ Florence, AUSL Toscana Ctr, Rare Dis Unit, Dept Hlth Sci,Sect Dermatol, Florence, Italy

[11] Guys & St Thomas NHS Fdn Trust, Dept Oral Med, London, England

[12] Newcastle Univ, Sch Dent Sci, Dept Oral Med, Newcastle Upon Tyne, Tyne & Wear, England

[13] Osped Classificato Cristo Re, Dept Dermatol, Rome, Italy

[14] Univ Amsterdam, Amsterdam Univ, Dept Otorhinolaryngol, Med Ctr, Amsterdam, Netherlands

[15] Univ Parma, Univ Hosp, Dept Med & Surg, Dermatol Unit, Parma, Italy

[16] Univ Klinikum Dusseldorf, Dept Ophthalmol, Dusseldorf, Germany

[17] Philipps Univ, Dept Dermatol & Allergol, Marburg, Germany

[18] Rouen Univ Hosp, Ctr Reference Malad Bulleuses Autoimmunes, Dept Dermatol, Rouen, France

[19] Normandie Univ, INSERM U1234, Rouen, France

[20] UCL, Eastman Dent Inst, Dept Oral Med, London, England

[21] Univ New South Wales, St George Hosp, Dept Dermatol, Sydney, NSW, Australia

[22] Aristotle Univ Thessaloniki, Papageorgiou Gen Hosp, Sch Med, Dept Dermatol 2,Autoimmune Bullous Dis Unit, Thessaloniki, Greece

[23] Univ Med Ctr Utrecht, Dept Dermatol & Allergol, Utrecht, Netherlands

[24] Guys & St Thomas NHS Fdn Trust, St Johns Inst Dermatol, London, England

[25] Kings Coll London, Ctr Host Microbiome Interact, Fac Dent Oral & Craniofacial Sci, London, England

[26] Grp Hosp Paris Seine St Denis, AP HP, Dept Dermatol, Bobigny, France

[27] Grp Hosp Paris Seine St Denis, AP HP, Referral Ctr Autoimmune Bullous Dis, Bobigny, France

[28] Univ Paris 13, Bobigny, France

[29] IRCCS, Bambino Gesu Childrens Hosp, Genet & Rare Dis Res Div, Genodermatosis Unit, Rome, Italy

来源：

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY | 2021年 / 35卷 / 10期

关键词：

EPIDERMOLYSIS-BULLOSA ACQUISITA; PULSED INTRAVENOUS CYCLOPHOSPHAMIDE; LINKED-IMMUNOSORBENT-ASSAY; SERRATION PATTERN-ANALYSIS; ORAL HYGIENE INSTRUCTIONS; TERM-FOLLOW-UP; DIRECT-IMMUNOFLUORESCENCE; PERIODONTAL STATUS; DESQUAMATIVE GINGIVITIS; IGG AUTOANTIBODIES;

D O I：

10.1111/jdv.17395

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

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.

引用

页码：1926 / 1948

页数：23

共 188 条

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