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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

A study of clinical, histopathological and direct immunofluorescence diagnosis in pemphigus group Utility of direct immunofluorescence.
Hrabovska Z, Jautova J, Hrabovsky V
Bratisl Lek Listy 2017;118(4):243-249. doi: 10.4149/BLL_2017_048. PMID: 28471236
Treatment of pemphigus.
Camisa C, Warner M
Dermatol Nurs 1998 Apr;10(2):115-8, 123-31. PMID: 9697444
Treatment of pemphigus.
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

Etiology

Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.
Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS
J Am Acad Dermatol 2021 Jun;84(6):1507-1519. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.11.075. PMID: 33684498
Pemphigus vegetans of the folds (intertriginous areas).
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
Necrotizing fasciitis caused by Cryptococcus neoformans in a patient with pemphigus vegetans.
Adachi M, Tsuruta D, Imanishi H, Ishii M, Kobayashi H
Clin Exp Dermatol 2009 Dec;34(8):e751-3. Epub 2009 Jul 29 doi: 10.1111/j.1365-2230.2009.03472.x. PMID: 19663839
Pemphigus.
Williams DS
J Insur Med 2004;36(3):267-8. PMID: 15495443
Childhood pemphigus.
Wananukul S, Pongprasit P
Int J Dermatol 1999 Jan;38(1):29-35. doi: 10.1046/j.1365-4362.1999.00618.x. PMID: 10065607

Diagnosis

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.
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
Pemphigus.
Williams DS
J Insur Med 2004;36(3):267-8. PMID: 15495443
Pyodermatitis-pyostomatitis vegetans.
Mehravaran M, Kemény L, Husz S, Korom I, Kiss M, Dobozy A
Br J Dermatol 1997 Aug;137(2):266-9. doi: 10.1046/j.1365-2133.1997.18181914.x. PMID: 9292079
Acantholytic acanthoma.
Brownstein MH
J Am Acad Dermatol 1988 Nov;19(5 Pt 1):783-6. doi: 10.1016/s0190-9622(88)70234-0. PMID: 2461398

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.
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
Pemphigus vegetans of the nose.
Sigmund GA, Oppenheimer R
Ear Nose Throat J 2012 Jan;91(1):E14-5. doi: 10.1177/014556131209100117. PMID: 22278872
Pemphigus.
Williams DS
J Insur Med 2004;36(3):267-8. PMID: 15495443
Pemphigus vegetans in a child.
Sillevis Smitt JH, Mulder TJ, Albeda FW, Van Nierop JC
Br J Dermatol 1992 Sep;127(3):289-91. doi: 10.1111/j.1365-2133.1992.tb00131.x. PMID: 1390175

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
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 vegetans of the folds (intertriginous areas).
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
Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study.
Zaraa I, Sellami A, Bouguerra C, Sellami MK, Chelly I, Zitouna M, Makni S, Hmida AB, Mokni M, Osman AB
J Eur Acad Dermatol Venereol 2011 Oct;25(10):1160-7. Epub 2010 Dec 29 doi: 10.1111/j.1468-3083.2010.03939.x. PMID: 21198951
Childhood pemphigus.
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

Dermoscopy of condyloma lata: An observational controlled study.
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
Captopril-induced pemphigus vegetans with Charcot-Leyden crystals.
Pinto GM, Lamarão P, Vale T
J Am Acad Dermatol 1992 Aug;27(2 Pt 2):281-4. doi: 10.1016/0190-9622(92)70180-n. PMID: 1387657
Charcot-Leyden crystals in pemphigus vegetans.
Kuo TT, Wang CN
J Cutan Pathol 1986 Jun;13(3):242-5. doi: 10.1111/j.1600-0560.1986.tb01651.x. PMID: 3734225
Further observations on pemphigus vegetans.
Schweers CA Jr, Hurt WC, Ploeg DE
Oral Surg Oral Med Oral Pathol 1969 Sep;28(3):442-50. doi: 10.1016/0030-4220(69)90241-2. PMID: 5257190
Observation on pemphigus vegetans.
Hurt WC
Oral Surg Oral Med Oral Pathol 1965 Oct;20(4):481-7. doi: 10.1016/0030-4220(65)90241-0. PMID: 5212794

Recent systematic reviews

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

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