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Mucous membrane pemphigoid

MedGen UID:
10619
•Concept ID:
C0030804
•
Disease or Syndrome
Synonyms: Benign Mucosal Pemphigoid; Benign Mucosal Pemphigoids; Benign Mucous Membrane Pemphigoid; Cicatricial Pemphigoid; Mucosal Pemphigoid, Benign; Mucosal Pemphigoids, Benign; Mucous Membrane Pemphigoid, Benign; Pemphigoid, Benign Mucosal; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Cicatricial; Pemphigoids, Benign Mucosal
SNOMED CT: Scarring pemphigoid (34250006); Mucous membrane pemphigoid (34250006); Benign mucosal pemphigoid (34250006); Benign mucous membrane pemphigoid (34250006); Cicatricial pemphigoid (34250006); Mucosynechia atrophic bullous dermatitis (34250006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018746
Orphanet: ORPHA46486

Definition

A rare autoimmune bullous skin disease characterized clinically by blistering of the mucous membranes followed by scarring, and immunologically characterized by IgG, IgA and/or C3 deposits on the epidermal basement membrane. The disease principally involves the oral mucosa, but may also affect ocular, pharyngolaryngeal, genital, and esophageal mucous membranes. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMucous membrane pemphigoid

Professional guidelines

PubMed

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II.
Schmidt E, Rashid H, Marzano AV, Lamberts A, Di Zenzo G, Diercks GFH, Alberti-Violetti S, Barry RJ, Borradori L, Caproni M, Carey B, Carrozzo M, Cianchini G, Corrà A, Dikkers FG, Feliciani C, Geerling G, Genovese G, Hertl M, Joly P, Meijer JM, Mercadante V, Murrell DF, Ormond M, Pas HH, Patsatsi A, Rauz S, van Rhijn BD, Roth M, Setterfield J, Zillikens D, C Prost, Zambruno G, Horváth B, Caux F
J Eur Acad Dermatol Venereol 2021 Oct;35(10):1926-1948. Epub 2021 Jul 26 doi: 10.1111/jdv.17395. PMID: 34309078Free PMC Article
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.
Patel PM, Jones VA, Murray TN, Amber KT
Am J Clin Dermatol 2020 Aug;21(4):557-565. doi: 10.1007/s40257-020-00513-3. PMID: 32180161
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article

Recent clinical studies

Etiology

Bullous Pemphigoid and Other Pemphigoid Dermatoses.
Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 4;57(10) doi: 10.3390/medicina57101061. PMID: 34684098Free PMC Article
European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.
Rashid H, Lamberts A, Borradori L, Alberti-Violetti S, Barry RJ, Caproni M, Carey B, Carrozzo M, Caux F, Cianchini G, Corrà A, Diercks GFH, Dikkers FG, Di Zenzo G, Feliciani C, Geerling G, Genovese G, Hertl M, Joly P, Marzano AV, Meijer JM, Mercadante V, Murrell DF, Ormond M, Pas HH, Patsatsi A, Prost C, Rauz S, van Rhijn BD, Roth M, Schmidt E, Setterfield J, Zambruno G, Zillikens D, Horváth B
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1750-1764. Epub 2021 Jul 10 doi: 10.1111/jdv.17397. PMID: 34245180Free PMC Article
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.
Patel PM, Jones VA, Murray TN, Amber KT
Am J Clin Dermatol 2020 Aug;21(4):557-565. doi: 10.1007/s40257-020-00513-3. PMID: 32180161
Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy.
Buonavoglia A, Leone P, Dammacco R, Di Lernia G, Petruzzi M, Bonamonte D, Vacca A, Racanelli V, Dammacco F
Autoimmun Rev 2019 Apr;18(4):349-358. Epub 2019 Feb 7 doi: 10.1016/j.autrev.2019.02.005. PMID: 30738958
Pemphigoid diseases.
Schmidt E, Zillikens D
Lancet 2013 Jan 26;381(9863):320-32. Epub 2012 Dec 11 doi: 10.1016/S0140-6736(12)61140-4. PMID: 23237497

Diagnosis

Mucous membrane pemphigoid.
Du G, Patzelt S, van Beek N, Schmidt E
Autoimmun Rev 2022 Apr;21(4):103036. Epub 2022 Jan 4 doi: 10.1016/j.autrev.2022.103036. PMID: 34995762
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.
Patel PM, Jones VA, Murray TN, Amber KT
Am J Clin Dermatol 2020 Aug;21(4):557-565. doi: 10.1007/s40257-020-00513-3. PMID: 32180161
Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy.
Buonavoglia A, Leone P, Dammacco R, Di Lernia G, Petruzzi M, Bonamonte D, Vacca A, Racanelli V, Dammacco F
Autoimmun Rev 2019 Apr;18(4):349-358. Epub 2019 Feb 7 doi: 10.1016/j.autrev.2019.02.005. PMID: 30738958
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article
Pemphigoid diseases.
Schmidt E, Zillikens D
Lancet 2013 Jan 26;381(9863):320-32. Epub 2012 Dec 11 doi: 10.1016/S0140-6736(12)61140-4. PMID: 23237497

Therapy

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II.
Schmidt E, Rashid H, Marzano AV, Lamberts A, Di Zenzo G, Diercks GFH, Alberti-Violetti S, Barry RJ, Borradori L, Caproni M, Carey B, Carrozzo M, Cianchini G, Corrà A, Dikkers FG, Feliciani C, Geerling G, Genovese G, Hertl M, Joly P, Meijer JM, Mercadante V, Murrell DF, Ormond M, Pas HH, Patsatsi A, Rauz S, van Rhijn BD, Roth M, Setterfield J, Zillikens D, C Prost, Zambruno G, Horváth B, Caux F
J Eur Acad Dermatol Venereol 2021 Oct;35(10):1926-1948. Epub 2021 Jul 26 doi: 10.1111/jdv.17395. PMID: 34309078Free PMC Article
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.
Patel PM, Jones VA, Murray TN, Amber KT
Am J Clin Dermatol 2020 Aug;21(4):557-565. doi: 10.1007/s40257-020-00513-3. PMID: 32180161
Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy.
Buonavoglia A, Leone P, Dammacco R, Di Lernia G, Petruzzi M, Bonamonte D, Vacca A, Racanelli V, Dammacco F
Autoimmun Rev 2019 Apr;18(4):349-358. Epub 2019 Feb 7 doi: 10.1016/j.autrev.2019.02.005. PMID: 30738958
Bullous Diseases.
Corbaux C, Joly P
Curr Probl Dermatol 2018;53:64-69. Epub 2017 Nov 7 doi: 10.1159/000478078. PMID: 29131038
Mucous membrane pemphigoid.
Xu HH, Werth VP, Parisi E, Sollecito TP
Dent Clin North Am 2013 Oct;57(4):611-30. Epub 2013 Aug 15 doi: 10.1016/j.cden.2013.07.003. PMID: 24034069Free PMC Article

Prognosis

European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.
Rashid H, Lamberts A, Borradori L, Alberti-Violetti S, Barry RJ, Caproni M, Carey B, Carrozzo M, Caux F, Cianchini G, Corrà A, Diercks GFH, Dikkers FG, Di Zenzo G, Feliciani C, Geerling G, Genovese G, Hertl M, Joly P, Marzano AV, Meijer JM, Mercadante V, Murrell DF, Ormond M, Pas HH, Patsatsi A, Prost C, Rauz S, van Rhijn BD, Roth M, Schmidt E, Setterfield J, Zambruno G, Zillikens D, Horváth B
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1750-1764. Epub 2021 Jul 10 doi: 10.1111/jdv.17397. PMID: 34245180Free PMC Article
Ocular mucous membrane pemphigoid: a review.
Taurone S, Spoletini M, Ralli M, Gobbi P, Artico M, Imre L, Czakò C, Kovàcs I, Greco A, Micera A
Immunol Res 2019 Jun;67(2-3):280-289. doi: 10.1007/s12026-019-09087-7. PMID: 31327149
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article
Pemphigoid diseases.
Schmidt E, Zillikens D
Lancet 2013 Jan 26;381(9863):320-32. Epub 2012 Dec 11 doi: 10.1016/S0140-6736(12)61140-4. PMID: 23237497
Anti-inflammatory treatment.
Fistarol SK, Itin PH
Curr Probl Dermatol 2011;40:58-70. Epub 2011 Feb 10 doi: 10.1159/000321051. PMID: 21325840

Clinical prediction guides

Autoimmunity against laminin 332.
Patzelt S, Schmidt E
Front Immunol 2023;14:1250115. Epub 2023 Aug 10 doi: 10.3389/fimmu.2023.1250115. PMID: 37638011Free PMC Article
S2k Guideline for the diagnosis and treatment of mucous membrane pemphigoid.
Hofmann SC, Günther C, Böckle BC, Didona D, Ehrchen J, Gaskins M, Geerling G, Gläser R, Hadaschik E, Hampl M, Haßkamp P, Jackowski J, Kiritsi D, Nast A, Pleyer U, Reichel C, Roth M, Schumann M, Sticherling M, Worm M, Zillikens D, Goebeler M, Schmidt E
J Dtsch Dermatol Ges 2022 Nov;20(11):1530-1550. Epub 2022 Nov 10 doi: 10.1111/ddg.14905. PMID: 36354061
Use of rituximab in the treatment of mucous membrane pemphigoid: An analytic review.
Farooq MM, Miloslavsky EM, Konikov N, Ahmed AR
Autoimmun Rev 2022 Aug;21(8):103119. Epub 2022 Jun 8 doi: 10.1016/j.autrev.2022.103119. PMID: 35688385
European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I.
Rashid H, Lamberts A, Borradori L, Alberti-Violetti S, Barry RJ, Caproni M, Carey B, Carrozzo M, Caux F, Cianchini G, Corrà A, Diercks GFH, Dikkers FG, Di Zenzo G, Feliciani C, Geerling G, Genovese G, Hertl M, Joly P, Marzano AV, Meijer JM, Mercadante V, Murrell DF, Ormond M, Pas HH, Patsatsi A, Prost C, Rauz S, van Rhijn BD, Roth M, Schmidt E, Setterfield J, Zambruno G, Zillikens D, Horváth B
J Eur Acad Dermatol Venereol 2021 Sep;35(9):1750-1764. Epub 2021 Jul 10 doi: 10.1111/jdv.17397. PMID: 34245180Free PMC Article
Mucous membrane pemphigoid and oral blistering diseases.
Carey B, Setterfield J
Clin Exp Dermatol 2019 Oct;44(7):732-739. Epub 2019 May 18 doi: 10.1111/ced.13996. PMID: 31102296

Recent systematic reviews

Brunsting-Perry pemphigoid: a systematic review.
Aromolo IF, Maronese CA, Moltrasio C, Genovese G, Marzano AV
Int J Dermatol 2022 Nov;61(11):1353-1358. Epub 2022 Jan 20 doi: 10.1111/ijd.16045. PMID: 35049061
Dental implants in patients suffering from autoimmune diseases: A systematic critical review.
Esimekara JO, Perez A, Courvoisier DS, Scolozzi P
J Stomatol Oral Maxillofac Surg 2022 Oct;123(5):e464-e473. Epub 2022 Jan 13 doi: 10.1016/j.jormas.2022.01.005. PMID: 35033725
Biologic treatment outcomes in mucous membrane pemphigoid: A systematic review.
Lytvyn Y, Rahat S, Mufti A, Witol A, Bagit A, Sachdeva M, Yeung J
J Am Acad Dermatol 2022 Jul;87(1):110-120. Epub 2021 Jan 7 doi: 10.1016/j.jaad.2020.12.056. PMID: 33422625
Periodontitis in oral pemphigus and pemphigoid: A systematic review of published studies.
Jascholt I, Lai O, Zillikens D, Kasperkiewicz M
J Am Acad Dermatol 2017 May;76(5):975-978.e3. Epub 2016 Dec 28 doi: 10.1016/j.jaad.2016.10.028. PMID: 28038889
Laryngeal mucous membrane pemphigoid: a systematic review and pooled-data analysis.
Higgins TS, Cohen JC, Sinacori JT
Laryngoscope 2010 Mar;120(3):529-36. doi: 10.1002/lary.20763. PMID: 20058317

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