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Pemphigus

MedGen UID:
45369
•Concept ID:
C0030807
•
Disease or Syndrome
Synonym: pemphigus
SNOMED CT: Pemphigus (65172003)
 
Monarch Initiative: MONDO:0006594

Definition

A blistering skin disorder. Morphologically it is characterized by acantholysis and intraepidermal blister formation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPemphigus

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
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

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
Peristomal Punctate Pemphigus.
Henning A, Bartenstein D, Kroshinsky D, Nazarian RM
Am J Dermatopathol 2021 Jul 1;43(7):510-513. doi: 10.1097/DAD.0000000000001845. PMID: 33156025
Pemphigus.
Schmidt E, Kasperkiewicz M, Joly P
Lancet 2019 Sep 7;394(10201):882-894. doi: 10.1016/S0140-6736(19)31778-7. PMID: 31498102
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
Drug-induced pemphigus.
Mutasim DF, Pelc NJ, Anhalt GJ
Dermatol Clin 1993 Jul;11(3):463-71. PMID: 8365032

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 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
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
Mechanisms of Disease: Pemphigus and Bullous Pemphigoid.
Hammers CM, Stanley JR
Annu Rev Pathol 2016 May 23;11:175-97. Epub 2016 Feb 22 doi: 10.1146/annurev-pathol-012615-044313. PMID: 26907530Free PMC Article
Pemphigus.
Santoro FA, Stoopler ET, Werth VP
Dent Clin North Am 2013 Oct;57(4):597-610. Epub 2013 Aug 12 doi: 10.1016/j.cden.2013.06.002. PMID: 24034068Free PMC Article

Therapy

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
Bruton's Tyrosine Kinase (BTK) Inhibitors and Autoimmune Diseases: Making Sense of BTK Inhibitor Specificity Profiles and Recent Clinical Trial Successes and Failures.
Ringheim GE, Wampole M, Oberoi K
Front Immunol 2021;12:662223. Epub 2021 Nov 3 doi: 10.3389/fimmu.2021.662223. PMID: 34803999Free PMC Article
Antigen-specific B cell depletion for precision therapy of mucosal pemphigus vulgaris.
Lee J, Lundgren DK, Mao X, Manfredo-Vieira S, Nunez-Cruz S, Williams EF, Assenmacher CA, Radaelli E, Oh S, Wang B, Ellebrecht CT, Fraietta JA, Milone MC, Payne AS
J Clin Invest 2020 Dec 1;130(12):6317-6324. doi: 10.1172/JCI138416. PMID: 32817591Free PMC Article
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

Immune serological diagnosis of pemphigus.
Solimani F, Meier K, Zimmer CL, Hashimoto T
Ital J Dermatol Venerol 2021 Apr;156(2):151-160. Epub 2020 Nov 23 doi: 10.23736/S2784-8671.20.06788-7. PMID: 33228340
Hailey-Hailey Disease: An Update Review with a Focus on Treatment Data.
Ben Lagha I, Ashack K, Khachemoune A
Am J Clin Dermatol 2020 Feb;21(1):49-68. doi: 10.1007/s40257-019-00477-z. PMID: 31595434
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
Management of Pemphigus Vulgaris.
Cholera M, Chainani-Wu N
Adv Ther 2016 Jun;33(6):910-58. Epub 2016 Jun 10 doi: 10.1007/s12325-016-0343-4. PMID: 27287854Free PMC Article
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

Integrative single-cell analysis reveals distinct adaptive immune signatures in the cutaneous lesions of pemphigus.
Xu C, Zhang T, Wang H, Zhu L, Ruan Y, Huang Z, Wang J, Zhu H, Huang C, Pan M
J Autoimmun 2024 Jan;142:103128. Epub 2023 Nov 6 doi: 10.1016/j.jaut.2023.103128. PMID: 37939532
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
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

Recent systematic reviews

Interventions for bullous pemphigoid.
Singh S, Kirtschig G, Anchan VN, Chi CC, Taghipour K, Boyle RJ, Murrell DF
Cochrane Database Syst Rev 2023 Aug 11;8(8):CD002292. doi: 10.1002/14651858.CD002292.pub4. PMID: 37572360Free PMC Article
Dupilumab in the treatment of genodermatosis: A systematic review.
Wu PC, Dai YX, Li CL, Chen CC, Chang YT, Ma SH
J Dtsch Dermatol Ges 2023 Jan;21(1):7-17. Epub 2023 Jan 19 doi: 10.1111/ddg.14924. PMID: 36657040
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
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Blistering diseases in neonates.
Zhao CY, Murrell DF
Curr Opin Pediatr 2016 Aug;28(4):500-6. doi: 10.1097/MOP.0000000000000381. PMID: 27386969

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