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Pemphigus vulgaris

MedGen UID:
10621
Concept ID:
C0030809
Disease or Syndrome
Synonym: Pemphigus Vulgaris
SNOMED CT: PV - Pemphigus vulgaris (49420001); Pemphigus vulgaris (49420001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0008219
Orphanet: ORPHA704

Definition

A rare autoimmune bullous skin diseases characterized by painful, flaccid blisters and erosions of the oral mucosa, predominantly involving the buccal area, and with or without extension to the epidermis. Mucosa of the larynx, oesophagus, conjunctiva, nose, genitalia and anus, are less frequently affected. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPemphigus vulgaris
Follow this link to review classifications for Pemphigus vulgaris in Orphanet.

Professional guidelines

PubMed

Joly P, Horvath B, Patsatsi Α, Uzun S, Bech R, Beissert S, Bergman R, Bernard P, Borradori L, Caproni M, Caux F, Cianchini G, Daneshpazhooh M, De D, Dmochowski M, Drenovska K, Ehrchen J, Feliciani C, Goebeler M, Groves R, Guenther C, Hofmann S, Ioannides D, Kowalewski C, Ludwig R, Lim YL, Marinovic B, Marzano AV, Mascaró JM Jr, Mimouni D, Murrell DF, Pincelli C, Squarcioni CP, Sárdy M, Setterfield J, Sprecher E, Vassileva S, Wozniak K, Yayli S, Zambruno G, Zillikens D, Hertl M, Schmidt E
J Eur Acad Dermatol Venereol 2020 Sep;34(9):1900-1913. Epub 2020 Aug 24 doi: 10.1111/jdv.16752. PMID: 32830877
Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, Sinha AA, Payne AS, Daneshpazhooh M, Eming R, Jonkman MF, Mimouni D, Borradori L, Kim SC, Yamagami J, Lehman JS, Saleh MA, Culton DA, Czernik A, Zone JJ, Fivenson D, Ujiie H, Wozniak K, Akman-Karakaş A, Bernard P, Korman NJ, Caux F, Drenovska K, Prost-Squarcioni C, Vassileva S, Feldman RJ, Cardones AR, Bauer J, Ioannides D, Jedlickova H, Palisson F, Patsatsi A, Uzun S, Yayli S, Zillikens D, Amagai M, Hertl M, Schmidt E, Aoki V, Grando SA, Shimizu H, Baum S, Cianchini G, Feliciani C, Iranzo P, Mascaró JM Jr, Kowalewski C, Hall R, Groves R, Harman KE, Marinkovich MP, Maverakis E, Werth VP
J Am Acad Dermatol 2020 Mar;82(3):575-585.e1. Epub 2018 Feb 10 doi: 10.1016/j.jaad.2018.02.021. PMID: 29438767Free PMC Article
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

Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 9;57(10) doi: 10.3390/medicina57101080. PMID: 34684117Free PMC Article
Yanovsky RL, McLeod M, Ahmed AR
Expert Rev Clin Immunol 2019 Oct;15(10):1061-1071. Epub 2019 Oct 10 doi: 10.1080/1744666X.2020.1672539. PMID: 31575295
Ellebrecht CT, Payne AS
JCI Insight 2017 Mar 9;2(5):e92021. doi: 10.1172/jci.insight.92021. PMID: 28289723Free PMC Article
Cirillo N, Cozzani E, Carrozzo M, Grando SA
Oral Dis 2012 Jul;18(5):442-58. Epub 2012 Feb 15 doi: 10.1111/j.1601-0825.2011.01899.x. PMID: 22335787
Troiano M, Lotti T
G Ital Dermatol Venereol 2009 Aug;144(4):495-9. PMID: 19755955

Diagnosis

Kianfar N, Daneshpazhooh M, Lehman JS
Mayo Clin Proc 2024 Apr;99(4):607-609. doi: 10.1016/j.mayocp.2023.11.006. PMID: 38569810
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
Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 9;57(10) doi: 10.3390/medicina57101080. PMID: 34684117Free PMC Article
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
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

Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J, Patel NJ, Zhang Y, Yue H, Bekker P, Heaney LG
Semin Arthritis Rheum 2022 Aug;55:152010. Epub 2022 Apr 15 doi: 10.1016/j.semarthrit.2022.152010. PMID: 35486995
Nayak V, Kini R, Rao PK, Shetty U
BMJ Case Rep 2020 May 13;13(5) doi: 10.1136/bcr-2020-235410. PMID: 32404374Free PMC Article
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
Hebert V, Joly P
Immunotherapy 2018 Jan;10(1):27-37. Epub 2017 Oct 24 doi: 10.2217/imt-2017-0104. PMID: 29064314
Liu V, Mackool BT
J Dermatolog Treat 2003 Dec;14(4):203-11. doi: 10.1080/09546630310016826. PMID: 14660264

Prognosis

Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J, Patel NJ, Zhang Y, Yue H, Bekker P, Heaney LG
Semin Arthritis Rheum 2022 Aug;55:152010. Epub 2022 Apr 15 doi: 10.1016/j.semarthrit.2022.152010. PMID: 35486995
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
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
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
Mutasim DF, Pelc NJ, Anhalt GJ
Dermatol Clin 1993 Jul;11(3):473-81. PMID: 8365033

Clinical prediction guides

Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J, Patel NJ, Zhang Y, Yue H, Bekker P, Heaney LG
Semin Arthritis Rheum 2022 Aug;55:152010. Epub 2022 Apr 15 doi: 10.1016/j.semarthrit.2022.152010. PMID: 35486995
Saleh MA, Saleh NA
Dermatol Ther 2022 Apr;35(4):e15354. Epub 2022 Feb 12 doi: 10.1111/dth.15354. PMID: 35108427
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jun;84(6):1523-1537. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2021.02.073. PMID: 33684497
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654

Recent systematic reviews

Carver CA, Kalesinskas M, Ahmed AR
Front Immunol 2023;14:1267668. Epub 2023 Oct 12 doi: 10.3389/fimmu.2023.1267668. PMID: 37901249Free PMC Article
Zhao L, Chen Y, Wang M
Dermatology 2023;239(4):514-522. Epub 2023 Mar 21 doi: 10.1159/000530121. PMID: 36944327
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
Seivright JR, Villa NM, De DR, Hsiao JL, Shi VY
Dermatol Ther 2022 Feb;35(2):e15234. Epub 2021 Dec 8 doi: 10.1111/dth.15234. PMID: 34825744
Aram K, Patil A, Goldust M, Rajabi F
Dermatol Ther 2021 Nov;34(6):e15113. Epub 2021 Sep 7 doi: 10.1111/dth.15113. PMID: 34453380Free PMC Article

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