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Benign mucous membrane pemphigoid

MedGen UID:
10619
•Concept ID:
C0030804
•
Disease or Syndrome
Synonym: Mucous membrane pemphigoid
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  
  • CROGVBenign mucous membrane pemphigoid

Professional guidelines

PubMed

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
Off-label use of biologicals in the management of inflammatory oral mucosal disease.
O'Neill ID
J Oral Pathol Med 2008 Nov;37(10):575-81. Epub 2008 Sep 1 doi: 10.1111/j.1600-0714.2008.00693.x. PMID: 18764859
Pharmacological management of recurrent oral mucosal ulceration.
Burgess JA, Johnson BD, Sommers E
Drugs 1990 Jan;39(1):54-65. doi: 10.2165/00003495-199039010-00005. PMID: 2178912

Recent clinical studies

Etiology

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
Spectrum of orocutaneous disease associations: Immune-mediated conditions.
Cizenski JD, Michel P, Watson IT, Frieder J, Wilder EG, Wright JM, Menter MA
J Am Acad Dermatol 2017 Nov;77(5):795-806. doi: 10.1016/j.jaad.2017.02.019. PMID: 29029901
Oral mucosal diseases: evaluation and management.
Stoopler ET, Sollecito TP
Med Clin North Am 2014 Nov;98(6):1323-52. Epub 2014 Sep 22 doi: 10.1016/j.mcna.2014.08.006. PMID: 25443679
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
Cicatricial pemphigoid-induced phimosis.
Fueston JC, Adams BB, Mutasim DF
J Am Acad Dermatol 2002 May;46(5 Suppl):S128-9. doi: 10.1067/mjd.2002.107967. PMID: 12004290

Diagnosis

Sarcoidosis-associated Cicatrizing Conjunctivitis.
Phylactou M
Ophthalmology 2023 Dec;130(12):1356. Epub 2023 Apr 12 doi: 10.1016/j.ophtha.2023.03.011. PMID: 37055290
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
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
Mucous membrane pemphigoid.
Chan LS
Clin Dermatol 2001 Nov-Dec;19(6):703-11. doi: 10.1016/s0738-081x(00)00196-6. PMID: 11705679

Therapy

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
Oesophageal cicatricial pemphigoid.
Diéguez Castillo C, Martín-Lagos Maldonado A, Del Moral Martínez M, Ruiz Escolano E
Gastroenterol Hepatol 2019 Jun-Jul;42(6):372-373. Epub 2018 Oct 3 doi: 10.1016/j.gastrohep.2018.08.007. PMID: 30292641
Ocular cicatricial pemphigoid.
Wang K, Seitzman G, Gonzales JA
Curr Opin Ophthalmol 2018 Nov;29(6):543-551. doi: 10.1097/ICU.0000000000000517. PMID: 30222656
Desquamative gingivitis: Diagnosis and treatment.
Maderal AD, Lee Salisbury P 3rd, Jorizzo JL
J Am Acad Dermatol 2018 May;78(5):851-861. doi: 10.1016/j.jaad.2017.04.1140. PMID: 29678379
Cicatrizing conjunctivitis.
Wright P
Trans Ophthalmol Soc U K (1962) 1986;105 ( Pt 1):1-17. PMID: 3520979

Prognosis

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
Spectrum of orocutaneous disease associations: Immune-mediated conditions.
Cizenski JD, Michel P, Watson IT, Frieder J, Wilder EG, Wright JM, Menter MA
J Am Acad Dermatol 2017 Nov;77(5):795-806. doi: 10.1016/j.jaad.2017.02.019. PMID: 29029901
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
Keratoprosthesis in autoimmune disease.
Ciralsky J, Papaliodis GN, Foster CS, Dohlman CH, Chodosh J
Ocul Immunol Inflamm 2010 Aug;18(4):275-80. doi: 10.3109/09273941003682300. PMID: 20662659
Cicatricial pemphigoid-induced phimosis.
Fueston JC, Adams BB, Mutasim DF
J Am Acad Dermatol 2002 May;46(5 Suppl):S128-9. doi: 10.1067/mjd.2002.107967. PMID: 12004290

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
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
Paraneoplastic cicatricial pemphigoid.
Setterfield J, Shirlaw PJ, Lazarova Z, Bryant BM, Bhogal BS, Harman K, Challacombe SJ, Black MM
Br J Dermatol 1999 Jul;141(1):127-31. doi: 10.1046/j.1365-2133.1999.02933.x. PMID: 10417528

Recent systematic reviews

Clinical Characteristics of Ocular Mucous Membrane Pemphigoid: A Systematic Review and Meta-Analysis.
Bocanegra-Oyola N, Pardo-Pizza D, Cifuentes-González C, Oliver-Hernández MV, Romero-Osorio MJ, Romero-Santos S, Parra-Tanoux D, Barragán AM, de-la-Torre A
Ocul Immunol Inflamm 2024 Dec;32(10):2388-2404. Epub 2024 Jul 29 doi: 10.1080/09273948.2024.2376746. PMID: 39074301
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
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
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
Ocular cicatricial pemphigoid.
Hingorani M, Lightman S
Curr Opin Allergy Clin Immunol 2006 Oct;6(5):373-8. doi: 10.1097/01.all.0000244799.33734.d4. PMID: 16954792

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