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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

Belimumab for the treatment of pemphigus.
Ruan J, Zhang X, Zheng M
Dermatol Ther 2022 Aug;35(8):e15621. Epub 2022 Jul 3 doi: 10.1111/dth.15621. PMID: 35671064
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
Treatment of pemphigus: an Indian perspective.
Kanwar AJ, Vinay K
Indian J Dermatol Venereol Leprol 2014 Jul-Aug;80(4):285-8. doi: 10.4103/0378-6323.136828. PMID: 25035350

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
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
Immunofluorescence of Autoimmune Bullous Diseases.
Diercks GF, Pas HH, Jonkman MF
Surg Pathol Clin 2017 Jun;10(2):505-512. Epub 2017 Mar 30 doi: 10.1016/j.path.2017.01.011. PMID: 28477893
Ocular pemphigus.
Daoud YJ, Cervantes R, Foster CS, Ahmed AR
J Am Acad Dermatol 2005 Oct;53(4):585-90. doi: 10.1016/j.jaad.2005.02.061. PMID: 16198777
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 Vegetans.
Radaschin DS, Laurentiu Tatu A
J Cutan Med Surg 2021 Jul-Aug;25(4):443. Epub 2020 Jun 30 doi: 10.1177/1203475420936656. PMID: 32602731
Editorial: Autoimmune Blistering Diseases.
Kowalewski C, Hashimoto T, Joly P
Front Immunol 2020;11:1614. Epub 2020 Jul 22 doi: 10.3389/fimmu.2020.01614. PMID: 32793239Free PMC Article
Pemphigus foliaceus.
Fariba G, Ayatollahi A, Hejazi S
Indian Pediatr 2012 Mar;49(3):240-1. PMID: 22484744
Paraneoplastic pemphigus.
Mutasim DF, Pelc NJ, Anhalt GJ
Dermatol Clin 1993 Jul;11(3):473-81. PMID: 8365033

Therapy

Pemphigus Vegetans.
Radaschin DS, Laurentiu Tatu A
J Cutan Med Surg 2021 Jul-Aug;25(4):443. Epub 2020 Jun 30 doi: 10.1177/1203475420936656. PMID: 32602731
Pemphigus vulgaris.
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
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
Rituximab in pemphigus.
Hebert V, Joly P
Immunotherapy 2018 Jan;10(1):27-37. Epub 2017 Oct 24 doi: 10.2217/imt-2017-0104. PMID: 29064314
Mycophenolate in dermatology.
Liu V, Mackool BT
J Dermatolog Treat 2003 Dec;14(4):203-11. doi: 10.1080/09546630310016826. PMID: 14660264

Prognosis

Painful mouth ulcers.
Rosa G, Piliang M
Cutis 2018 May;101(5):327;341;342;345. PMID: 29894533
Treatment of Autoimmune Bullous Disorders in Pregnancy.
Kushner CJ, Concha JSS, Werth VP
Am J Clin Dermatol 2018 Jun;19(3):391-403. doi: 10.1007/s40257-018-0342-0. PMID: 29392620
Paraneoplastic pemphigus.
Anhalt GJ
J Investig Dermatol Symp Proc 2004 Jan;9(1):29-33. doi: 10.1111/j.1087-0024.2004.00832.x. PMID: 14870982
Autoimmune blistering skin diseases.
Cotell S, Robinson ND, Chan LS
Am J Emerg Med 2000 May;18(3):288-99. doi: 10.1016/s0735-6757(00)90124-4. PMID: 10830686
Drug-induced pemphigus.
Brenner S, Bialy-Golan A, Ruocco V
Clin Dermatol 1998 May-Jun;16(3):393-7. doi: 10.1016/s0738-081x(98)00010-8. PMID: 9642533

Clinical prediction guides

Intraepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
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
Therapy of autoimmune mouth bullous disease: 2020 review.
Giannetti L, Murri Dello Diago A
Dermatol Ther 2021 Jan;34(1):e14376. Epub 2020 Oct 19 doi: 10.1111/dth.14376. PMID: 33029891
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
Hailey-Hailey disease: A fold (intertriginous) dermatosis.
Engin B, Kutlubay Z, Çelik U, Serdaroğlu S, Tüzün Y
Clin Dermatol 2015 Jul-Aug;33(4):452-5. Epub 2015 Apr 8 doi: 10.1016/j.clindermatol.2015.04.006. PMID: 26051060
Ocular pemphigus.
Daoud YJ, Cervantes R, Foster CS, Ahmed AR
J Am Acad Dermatol 2005 Oct;53(4):585-90. doi: 10.1016/j.jaad.2005.02.061. PMID: 16198777

Recent systematic reviews

Bullous Dermatoses and Quality of Life: A Summary of Tools to Assess Psychosocial Health.
Riopelle A, Lake E
Cutis 2022 Jan;109(1):E14-E19. doi: 10.12788/cutis.0439. PMID: 35180062
Intralesional biologics for inflammatory dermatoses: A systematic review.
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
COVID-19 and exacerbation of dermatological diseases: A review of the available literature.
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
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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