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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
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
Differential diagnosis and management of oral ulcers.
Siu A, Landon K, Ramos DM
Semin Cutan Med Surg 2015 Dec;34(4):171-7. doi: 10.12788/j.sder.2015.0170. PMID: 26650694

Recent clinical studies

Etiology

Autoimmune bullous dermatoses.
Holtsche MM, Boch K, Schmidt E
J Dtsch Dermatol Ges 2023 Apr;21(4):405-412. doi: 10.1111/ddg.15046. PMID: 37070500
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
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
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

Vesiculobullous Lesions of the Oral Cavity.
Alramadhan SA, Islam MN
Oral Maxillofac Surg Clin North Am 2023 May;35(2):203-217. doi: 10.1016/j.coms.2022.10.006. PMID: 37019505
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
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
Differential diagnosis and management of oral ulcers.
Siu A, Landon K, Ramos DM
Semin Cutan Med Surg 2015 Dec;34(4):171-7. doi: 10.12788/j.sder.2015.0170. PMID: 26650694
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

Autoimmune bullous dermatoses.
Holtsche MM, Boch K, Schmidt E
J Dtsch Dermatol Ges 2023 Apr;21(4):405-412. doi: 10.1111/ddg.15046. PMID: 37070500
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
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

Tissue Remodeling in Ocular Mucous Membrane Pemphigoid.
Di Zazzo A, Cutrupi F, De Antoniis MG, Ricci M, Esposito G, Antonini M, Coassin M, Micera A, Perrella E, Bonini S
Invest Ophthalmol Vis Sci 2023 Dec 1;64(15):17. doi: 10.1167/iovs.64.15.17. PMID: 38095906Free PMC Article
Conjunctival transcriptomics in ocular mucous membrane pemphigoid.
Panthagani J, Suleiman K, Vincent RC, Ong HS, Wallace GR, Rauz S
Ocul Surf 2023 Oct;30:142-149. Epub 2023 Sep 9 doi: 10.1016/j.jtos.2023.09.005. PMID: 37690517
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
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

Pemphigus vulgaris and mucous membrane pemphigoid: A systematic review of clinical manifestations, diagnosis, and treatment.
Morais HGF, Costa CSO, Medeiros MRS, Carlan LM, Gonzaga AKG, Freitas RA, da Silveira ÉJD, de Oliveira PT
J Stomatol Oral Maxillofac Surg 2024 Oct;125(5S2):101960. Epub 2024 Jul 1 doi: 10.1016/j.jormas.2024.101960. PMID: 38960026
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

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