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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV).
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
Diagnosis and management of pemphigus: Recommendations of an international panel of experts.
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
Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation, investigations and management.
Melchionda V, Harman KE
Clin Exp Dermatol 2019 Oct;44(7):740-746. Epub 2019 Aug 4 doi: 10.1111/ced.14041. PMID: 31378971

Recent clinical studies

Etiology

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open-label feasibility trial.
Goebeler M, Bata-Csörgő Z, De Simone C, Didona B, Remenyik E, Reznichenko N, Stoevesandt J, Ward ES, Parys W, de Haard H, Dupuy P, Verheesen P, Schmidt E, Joly P; ARGX-113-1701 Investigator Study Group
Br J Dermatol 2022 Mar;186(3):429-439. Epub 2021 Nov 28 doi: 10.1111/bjd.20782. PMID: 34608631
An Updated Review of Pemphigus Diseases.
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
Rituximab versus Mycophenolate Mofetil in Patients with Pemphigus Vulgaris.
Werth VP, Joly P, Mimouni D, Maverakis E, Caux F, Lehane P, Gearhart L, Kapre A, Pordeli P, Chen DM; PEMPHIX Study Group
N Engl J Med 2021 Jun 17;384(24):2295-2305. Epub 2021 May 19 doi: 10.1056/NEJMoa2028564. PMID: 34097368
Pemphigus Vulgaris: A Retrospective Cohort Study of Clinical Features, Treatments, and Outcomes.
Cura MJ, Torre AC, Cueto Sarmiento KY, Bollea Garlatti ML, Riganti J, Puga MC, Mazzuoccolo LD
Actas Dermosifiliogr (Engl Ed) 2020 Jun;111(5):398-407. Epub 2020 May 25 doi: 10.1016/j.ad.2019.10.004. PMID: 32466985
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654

Diagnosis

An Updated Review of Pemphigus Diseases.
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
Pemphigus Vulgaris: A Retrospective Cohort Study of Clinical Features, Treatments, and Outcomes.
Cura MJ, Torre AC, Cueto Sarmiento KY, Bollea Garlatti ML, Riganti J, Puga MC, Mazzuoccolo LD
Actas Dermosifiliogr (Engl Ed) 2020 Jun;111(5):398-407. Epub 2020 May 25 doi: 10.1016/j.ad.2019.10.004. PMID: 32466985
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
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
Oral manifestations of systemic disease.
Chi AC, Neville BW, Krayer JW, Gonsalves WC
Am Fam Physician 2010 Dec 1;82(11):1381-8. PMID: 21121523

Therapy

The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time.
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
Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open-label feasibility trial.
Goebeler M, Bata-Csörgő Z, De Simone C, Didona B, Remenyik E, Reznichenko N, Stoevesandt J, Ward ES, Parys W, de Haard H, Dupuy P, Verheesen P, Schmidt E, Joly P; ARGX-113-1701 Investigator Study Group
Br J Dermatol 2022 Mar;186(3):429-439. Epub 2021 Nov 28 doi: 10.1111/bjd.20782. PMID: 34608631
Pemphigus Vulgaris: A Retrospective Cohort Study of Clinical Features, Treatments, and Outcomes.
Cura MJ, Torre AC, Cueto Sarmiento KY, Bollea Garlatti ML, Riganti J, Puga MC, Mazzuoccolo LD
Actas Dermosifiliogr (Engl Ed) 2020 Jun;111(5):398-407. Epub 2020 May 25 doi: 10.1016/j.ad.2019.10.004. PMID: 32466985
Rituximab: A Review in Pemphigus Vulgaris.
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Prognosis

The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time.
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
Pemphigus Vulgaris: A Retrospective Cohort Study of Clinical Features, Treatments, and Outcomes.
Cura MJ, Torre AC, Cueto Sarmiento KY, Bollea Garlatti ML, Riganti J, Puga MC, Mazzuoccolo LD
Actas Dermosifiliogr (Engl Ed) 2020 Jun;111(5):398-407. Epub 2020 May 25 doi: 10.1016/j.ad.2019.10.004. PMID: 32466985
Rituximab: A Review in Pemphigus Vulgaris.
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
IgA pemphigus.
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, Imamura K, Kobayashi H, Karashima T, Nakama T, Dainichi T, Hashimoto T
Clin Dermatol 2011 Jul-Aug;29(4):437-42. doi: 10.1016/j.clindermatol.2011.01.014. PMID: 21679872

Clinical prediction guides

The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time.
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
Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open-label feasibility trial.
Goebeler M, Bata-Csörgő Z, De Simone C, Didona B, Remenyik E, Reznichenko N, Stoevesandt J, Ward ES, Parys W, de Haard H, Dupuy P, Verheesen P, Schmidt E, Joly P; ARGX-113-1701 Investigator Study Group
Br J Dermatol 2022 Mar;186(3):429-439. Epub 2021 Nov 28 doi: 10.1111/bjd.20782. PMID: 34608631
Rituximab versus Mycophenolate Mofetil in Patients with Pemphigus Vulgaris.
Werth VP, Joly P, Mimouni D, Maverakis E, Caux F, Lehane P, Gearhart L, Kapre A, Pordeli P, Chen DM; PEMPHIX Study Group
N Engl J Med 2021 Jun 17;384(24):2295-2305. Epub 2021 May 19 doi: 10.1056/NEJMoa2028564. PMID: 34097368
Rituximab: A Review in Pemphigus Vulgaris.
Frampton JE
Am J Clin Dermatol 2020 Feb;21(1):149-156. doi: 10.1007/s40257-019-00497-9. PMID: 31838645
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654

Recent systematic reviews

Pregnancy in pemphigus vulgaris: A systematic review.
Lan Y, Zhang H, Jin H
Am J Reprod Immunol 2024 Jan;91(1):e13813. doi: 10.1111/aji.13813. PMID: 38282607
The Global Incidence Rate of Pemphigus Vulgaris: A Systematic Review and Meta-Analysis.
Zhao L, Chen Y, Wang M
Dermatology 2023;239(4):514-522. Epub 2023 Mar 21 doi: 10.1159/000530121. PMID: 36944327
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
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
Treatment of pemphigus vulgaris and pemphigus foliaceus: a systematic review and meta-analysis.
Atzmony L, Hodak E, Gdalevich M, Rosenbaum O, Mimouni D
Am J Clin Dermatol 2014 Dec;15(6):503-15. doi: 10.1007/s40257-014-0101-9. PMID: 25403548

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