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Pemphigus foliaceus(PF)

MedGen UID:
75513
•Concept ID:
C0263313
•
Disease or Syndrome
Synonym: PF
SNOMED CT: PF - Pemphigus foliaceous (35154004); Pemphigus foliaceous (35154004); Pemphigus foliaceus (35154004)
 
Monarch Initiative: MONDO:0019324
Orphanet: ORPHA79481

Definition

A rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

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
Pemphigus: Pathogenesis to Treatment.
DiMarco C
R I Med J (2013) 2016 Dec 1;99(12):28-31. PMID: 27902996

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
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.
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
Pemphigus.
Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M
Nat Rev Dis Primers 2017 May 11;3:17026. doi: 10.1038/nrdp.2017.26. PMID: 28492232Free PMC Article

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
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.
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
Pemphigus.
Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M
Nat Rev Dis Primers 2017 May 11;3:17026. doi: 10.1038/nrdp.2017.26. PMID: 28492232Free 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
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.
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.
Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M
Nat Rev Dis Primers 2017 May 11;3:17026. doi: 10.1038/nrdp.2017.26. PMID: 28492232Free PMC Article
Immunoadsorption in dermatology.
Meyersburg D, Schmidt E, Kasperkiewicz M, Zillikens D
Ther Apher Dial 2012 Aug;16(4):311-20. Epub 2012 May 15 doi: 10.1111/j.1744-9987.2012.01075.x. PMID: 22817118

Prognosis

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
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
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
Penicillamine-induced bullous dermatoses.
Bialy-Golan A, Brenner S
J Am Acad Dermatol 1996 Nov;35(5 Pt 1):732-42. doi: 10.1016/s0190-9622(96)90729-x. PMID: 8912569

Clinical prediction guides

Current biologics in treatment of pemphigus foliaceus: a systematic review.
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
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 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
Staphylococcal epidermolysins.
Prévost G, Couppié P, Monteil H
Curr Opin Infect Dis 2003 Apr;16(2):71-6. doi: 10.1097/00001432-200304000-00002. PMID: 12734438

Recent systematic reviews

Current biologics in treatment of pemphigus foliaceus: a systematic review.
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
The role of adjuvant therapy in pemphigus: A systematic review and meta-analysis.
Atzmony L, Hodak E, Leshem YA, Rosenbaum O, Gdalevich M, Anhalt GJ, Mimouni D
J Am Acad Dermatol 2015 Aug;73(2):264-71. Epub 2015 Jun 16 doi: 10.1016/j.jaad.2015.04.038. PMID: 26088689
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
Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review.
Singh S
Indian J Dermatol Venereol Leprol 2011 Jul-Aug;77(4):456-69. doi: 10.4103/0378-6323.82400. PMID: 21727693
A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus.
Martin LK, Werth VP, Villaneuva EV, Murrell DF
J Am Acad Dermatol 2011 May;64(5):903-8. Epub 2011 Feb 25 doi: 10.1016/j.jaad.2010.04.039. PMID: 21353333Free PMC Article

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