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Bullous pemphigoid

MedGen UID:
10620
Concept ID:
C0030805
Disease or Syndrome
Synonyms: Benign Pemphigus; Old Age Pemphigus; Parapemphigus; Senile Dermatitis Herpetiformis
SNOMED CT: BP - Bullous pemphigoid (77090002); Bullous pemphigoid (77090002); Pemphigoid (86142006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019082
Orphanet: ORPHA703

Definition

A rare autoimmune bullous skin disease characterized by acquired, subepidermal tense bullae occurring on normal of inflamed skin and that is typically widespread (occurring in the flexor regions of the proximal arms and legs, in the armpits, groin and the abdomen) and often associated with pruritus. The evolution is typically chronic with spontaneous exacerbations and remission. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBullous pemphigoid
Follow this link to review classifications for Bullous pemphigoid in Orphanet.

Professional guidelines

PubMed

Borradori L, Van Beek N, Feliciani C, Tedbirt B, Antiga E, Bergman R, Böckle BC, Caproni M, Caux F, Chandran NS, Cianchini G, Daneshpazhooh M, De D, Didona D, Di Zenzo GM, Dmochowski M, Drenovska K, Ehrchen J, Goebeler M, Groves R, Günther C, Horvath B, Hertl M, Hofmann S, Ioannides D, Itzlinger-Monshi B, Jedličková J, Kowalewski C, Kridin K, Lim YL, Marinovic B, Marzano AV, Mascaro JM, Meijer JM, Murrell D, Patsatsi K, Pincelli C, Prost C, Rappersberger K, Sárdy M, Setterfield J, Shahid M, Sprecher E, Tasanen K, Uzun S, Vassileva S, Vestergaard K, Vorobyev A, Vujic I, Wang G, Wozniak K, Yayli S, Zambruno G, Zillikens D, Schmidt E, Joly P
J Eur Acad Dermatol Venereol 2022 Oct;36(10):1689-1704. Epub 2022 Jun 29 doi: 10.1111/jdv.18220. PMID: 35766904
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089

Recent clinical studies

Etiology

Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 4;57(10) doi: 10.3390/medicina57101061. PMID: 34684098Free PMC Article
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Miyamoto D, Santi CG, Aoki V, Maruta CW
An Bras Dermatol 2019 Mar-Apr;94(2):133-146. Epub 2019 May 9 doi: 10.1590/abd1806-4841.20199007. PMID: 31090818Free PMC Article
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089
Schmidt E, Zillikens D
Lancet 2013 Jan 26;381(9863):320-32. Epub 2012 Dec 11 doi: 10.1016/S0140-6736(12)61140-4. PMID: 23237497

Diagnosis

Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 4;57(10) doi: 10.3390/medicina57101061. PMID: 34684098Free PMC Article
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Verheyden MJ, Bilgic A, Murrell DF
Acta Derm Venereol 2020 Aug 17;100(15):adv00224. doi: 10.2340/00015555-3457. PMID: 32176310Free PMC Article
Miyamoto D, Santi CG, Aoki V, Maruta CW
An Bras Dermatol 2019 Mar-Apr;94(2):133-146. Epub 2019 May 9 doi: 10.1590/abd1806-4841.20199007. PMID: 31090818Free PMC Article
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

Therapy

Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Cao P, Xu W, Zhang L
Front Immunol 2022;13:928621. Epub 2022 Jun 13 doi: 10.3389/fimmu.2022.928621. PMID: 35769474Free PMC Article
Huber R, Wong A
Skin Therapy Lett 2020 Nov;25(5):7-11. PMID: 33196157
Miyamoto D, Santi CG, Aoki V, Maruta CW
An Bras Dermatol 2019 Mar-Apr;94(2):133-146. Epub 2019 May 9 doi: 10.1590/abd1806-4841.20199007. PMID: 31090818Free PMC Article
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

Oren-Shabtai M, Mimouni D, Nosrati A, Atzmony L, Kaplan B, Barzilai A, Baum S
Front Immunol 2023;14:1157250. Epub 2023 Apr 27 doi: 10.3389/fimmu.2023.1157250. PMID: 37180101Free PMC Article
Stoj V, Lu J
Clin Dermatol 2022 Mar-Apr;40(2):156-165. Epub 2021 Nov 19 doi: 10.1016/j.clindermatol.2021.10.009. PMID: 34808244
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Sibaud V
Am J Clin Dermatol 2018 Jun;19(3):345-361. doi: 10.1007/s40257-017-0336-3. PMID: 29256113
Schmidt E, Zillikens D
Lancet 2013 Jan 26;381(9863):320-32. Epub 2012 Dec 11 doi: 10.1016/S0140-6736(12)61140-4. PMID: 23237497

Clinical prediction guides

Zhao L, Wang Q, Liang G, Zhou Y, Yiu N, Yang B, Zhang G, Li W, Feng S, Shang P, Chen X, Zhu X, Zheng J, Pan M, Wang M
JAMA Dermatol 2023 Sep 1;159(9):953-960. doi: 10.1001/jamadermatol.2023.2428. PMID: 37531116Free PMC Article
Cao P, Xu W, Zhang L
Front Immunol 2022;13:928621. Epub 2022 Jun 13 doi: 10.3389/fimmu.2022.928621. PMID: 35769474Free PMC Article
Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J, Patel NJ, Zhang Y, Yue H, Bekker P, Heaney LG
Semin Arthritis Rheum 2022 Aug;55:152010. Epub 2022 Apr 15 doi: 10.1016/j.semarthrit.2022.152010. PMID: 35486995
Cohen PR
Medicina (Kaunas) 2021 Apr 20;57(4) doi: 10.3390/medicina57040398. PMID: 33924249Free PMC Article
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089

Recent systematic reviews

Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Cao P, Xu W, Zhang L
Front Immunol 2022;13:928621. Epub 2022 Jun 13 doi: 10.3389/fimmu.2022.928621. PMID: 35769474Free PMC Article
Aromolo IF, Maronese CA, Moltrasio C, Genovese G, Marzano AV
Int J Dermatol 2022 Nov;61(11):1353-1358. Epub 2022 Jan 20 doi: 10.1111/ijd.16045. PMID: 35049061
Verheyden MJ, Bilgic A, Murrell DF
Acta Derm Venereol 2020 Aug 17;100(15):adv00224. doi: 10.2340/00015555-3457. PMID: 32176310Free PMC Article
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

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