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Pemphigus vegetans

MedGen UID:
537814
Concept ID:
C0263316
Disease or Syndrome
Synonym: pemphigus vegetans
SNOMED CT: Pemphigus vegetans (81285006)
 
Monarch Initiative: MONDO:0019322
Orphanet: ORPHA79479

Definition

A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

Mesquita Kde C, Igreja AC
An Bras Dermatol 2012 Mar-Apr;87(2):339. doi: 10.1590/s0365-05962012000200034. PMID: 22570054
Camisa C, Warner M
Dermatol Nurs 1998 Apr;10(2):115-8, 123-31. PMID: 9697444
Piamphongsant T, Ophaswongse S
Int J Dermatol 1991 Feb;30(2):139-46. doi: 10.1111/j.1365-4362.1991.tb04229.x. PMID: 2001906

Recent clinical studies

Therapy

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
Porro AM, Seque CA, Ferreira MCC, Enokihara MMSES
An Bras Dermatol 2019 Jul 29;94(3):264-278. doi: 10.1590/abd1806-4841.20199011. PMID: 31365654Free PMC Article
Ruocco V, Ruocco E, Caccavale S, Gambardella A, Lo Schiavo A
Clin Dermatol 2015 Jul-Aug;33(4):471-6. Epub 2015 Apr 8 doi: 10.1016/j.clindermatol.2015.04.011. PMID: 26051064
Wananukul S, Pongprasit P
Int J Dermatol 1999 Jan;38(1):29-35. doi: 10.1046/j.1365-4362.1999.00618.x. PMID: 10065607
Camisa C, Warner M
Dermatol Nurs 1998 Apr;10(2):115-8, 123-31. PMID: 9697444

Prognosis

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
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
Sossau D, Kofler L, Schaller M, Yazdi A
Eur J Dermatol 2019 Apr 1;29(2):209-210. doi: 10.1684/ejd.2018.3407. PMID: 30977725
Ruocco V, Ruocco E, Caccavale S, Gambardella A, Lo Schiavo A
Clin Dermatol 2015 Jul-Aug;33(4):471-6. Epub 2015 Apr 8 doi: 10.1016/j.clindermatol.2015.04.011. PMID: 26051064
Wananukul S, Pongprasit P
Int J Dermatol 1999 Jan;38(1):29-35. doi: 10.1046/j.1365-4362.1999.00618.x. PMID: 10065607

Clinical prediction guides

Yamamoto T
Exp Dermatol 2024 Jan;33(1):e14931. Epub 2023 Sep 11 doi: 10.1111/exd.14931. PMID: 37694984
Errichetti E, Lallas A, Galvan A
Australas J Dermatol 2023 Nov;64(4):e381-e383. Epub 2023 Sep 14 doi: 10.1111/ajd.14155. PMID: 37706598
Lotti R, Atene CG, Marconi A, Di Rocco G, Reggiani Bonetti L, Zanocco Marani T, Pincelli C
Front Immunol 2019;10:1387. Epub 2019 Jun 19 doi: 10.3389/fimmu.2019.01387. PMID: 31275323Free PMC Article
von Köckritz A, Ständer S, Zeidler C, Metze D, Luger T, Bonsmann G
J Eur Acad Dermatol Venereol 2017 Jan;31(1):85-88. Epub 2016 Sep 7 doi: 10.1111/jdv.13779. PMID: 27600093
Wang H, Qiao S, Zhang X, Liu C
Am J Med Sci 2013 Feb;345(2):168-71. doi: 10.1097/MAJ.0b013e31826c5a82. PMID: 23138121

Recent systematic reviews

Singh S
Indian J Dermatol Venereol Leprol 2011 Jul-Aug;77(4):456-69. doi: 10.4103/0378-6323.82400. PMID: 21727693

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