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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

Keratoprosthesis in dry eye disease.
Sharma S, Donthineni PR, Iyer G, Chodosh J, de la Paz MF, Maskati Q, Srinivasan B, Agarwal S, Basu S, Shanbhag SS
Indian J Ophthalmol 2023 Apr;71(4):1154-1166. doi: 10.4103/IJO.IJO_2817_22. PMID: 37026247Free PMC Article
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
Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.
Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS
J Am Acad Dermatol 2021 Jul;85(1):1-14. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.11.076. PMID: 33684496
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
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

Diagnosis

Keratoprosthesis in dry eye disease.
Sharma S, Donthineni PR, Iyer G, Chodosh J, de la Paz MF, Maskati Q, Srinivasan B, Agarwal S, Basu S, Shanbhag SS
Indian J Ophthalmol 2023 Apr;71(4):1154-1166. doi: 10.4103/IJO.IJO_2817_22. PMID: 37026247Free PMC Article
Mucocutaneous Diseases.
Kuten-Shorrer M, Menon RS, Lerman MA
Dent Clin North Am 2020 Jan;64(1):139-162. Epub 2019 Oct 21 doi: 10.1016/j.cden.2019.08.009. PMID: 31735224
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
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

Therapy

Keratoprosthesis in dry eye disease.
Sharma S, Donthineni PR, Iyer G, Chodosh J, de la Paz MF, Maskati Q, Srinivasan B, Agarwal S, Basu S, Shanbhag SS
Indian J Ophthalmol 2023 Apr;71(4):1154-1166. doi: 10.4103/IJO.IJO_2817_22. PMID: 37026247Free PMC Article
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
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
Update on ocular cicatricial pemphigoid and emerging treatments.
Queisi MM, Zein M, Lamba N, Meese H, Foster CS
Surv Ophthalmol 2016 May-Jun;61(3):314-7. Epub 2015 Dec 19 doi: 10.1016/j.survophthal.2015.12.007. PMID: 26708362

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
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
The blistering diseases.
Scott JE, Ahmed AR
Med Clin North Am 1998 Nov;82(6):1239-83. doi: 10.1016/s0025-7125(05)70415-6. PMID: 9889748

Clinical prediction guides

Ocular Cicatricial Pemphigoid in Young Patients.
Jinagal J, Gupta B, Hanumanthu V, Chatterjee D, Limbu S, Malhotra C, Gupta A, Jain AK, Yangzes S, Mahajan R, Handa S, Pandav SS, De D
Cornea 2024 Aug 1;43(8):1015-1021. Epub 2024 Apr 26 doi: 10.1097/ICO.0000000000003547. PMID: 38692674
Platelet-rich fibrin a new approach in management of persistent oral ulcers in blistering skin diseases.
Ahmed EM, Shafik S, El Saadany B
Wound Repair Regen 2022 Jul;30(4):491-497. Epub 2022 Jun 3 doi: 10.1111/wrr.13020. PMID: 35589608
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
Signaling lipids as diagnostic biomarkers for ocular surface cicatrizing conjunctivitis.
Di Zazzo A, Yang W, Coassin M, Micera A, Antonini M, Piccinni F, De Piano M, Kohler I, Harms AC, Hankemeier T, Boinini S, Mashaghi A
J Mol Med (Berl) 2020 May;98(5):751-760. Epub 2020 Apr 20 doi: 10.1007/s00109-020-01907-w. PMID: 32313985Free PMC Article
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

Oral mucosal toxicities induced by immune checkpoint inhibitors: Clinical features and algorithm management.
Vigarios E, Sibaud V
Ann Dermatol Venereol 2023 Jun;150(2):83-88. Epub 2023 Mar 21 doi: 10.1016/j.annder.2022.11.012. PMID: 36935341
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
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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