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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV).
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
Eosinophilic Dermatoses: Recognition and Management.
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Bullous Pemphigoid: A Review of its Diagnosis, Associations and Treatment.
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

Bullous Pemphigoid and Other Pemphigoid Dermatoses.
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
Bullous Pemphigoid: Trigger and Predisposing Factors.
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
Bullous pemphigoid.
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
Bullous Pemphigoid: A Review of its Diagnosis, Associations and Treatment.
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089
Pemphigoid diseases.
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

Bullous Pemphigoid and Other Pemphigoid Dermatoses.
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
Bullous Pemphigoid: Trigger and Predisposing Factors.
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
A Systematic Review of Drug-Induced Pemphigoid.
Verheyden MJ, Bilgic A, Murrell DF
Acta Derm Venereol 2020 Aug 17;100(15):adv00224. doi: 10.2340/00015555-3457. PMID: 32176310Free PMC Article
Bullous pemphigoid.
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
Mechanisms of Disease: Pemphigus and Bullous Pemphigoid.
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

Dupilumab in Inflammatory Skin Diseases: A Systematic Review.
Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Rituximab, Omalizumab, and Dupilumab Treatment Outcomes in Bullous Pemphigoid: A Systematic Review.
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
Nicotinamide: An Update and Review of Safety & Differences from Niacin.
Huber R, Wong A
Skin Therapy Lett 2020 Nov;25(5):7-11. PMID: 33196157
Bullous pemphigoid.
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
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
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

Nutrition and bullous diseases.
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
Bullous Pemphigoid: Trigger and Predisposing Factors.
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
Bullous pemphigoid in adolescence.
Patsatsi A, Kyriakou A, Werth VP
Pediatr Dermatol 2019 Mar;36(2):184-188. Epub 2018 Dec 19 doi: 10.1111/pde.13717. PMID: 30569520
Dermatologic Reactions to Immune Checkpoint Inhibitors : Skin Toxicities and Immunotherapy.
Sibaud V
Am J Clin Dermatol 2018 Jun;19(3):345-361. doi: 10.1007/s40257-017-0336-3. PMID: 29256113
Pemphigoid diseases.
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

Evaluation of Dupilumab in Patients With Bullous Pemphigoid.
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
Rituximab, Omalizumab, and Dupilumab Treatment Outcomes in Bullous Pemphigoid: A Systematic Review.
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
The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time.
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
Dyshidrosiform Bullous Pemphigoid.
Cohen PR
Medicina (Kaunas) 2021 Apr 20;57(4) doi: 10.3390/medicina57040398. PMID: 33924249Free PMC Article
Bullous Pemphigoid: A Review of its Diagnosis, Associations and Treatment.
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

Dupilumab in Inflammatory Skin Diseases: A Systematic Review.
Olbrich H, Sadik CD, Ludwig RJ, Thaçi D, Boch K
Biomolecules 2023 Mar 31;13(4) doi: 10.3390/biom13040634. PMID: 37189381Free PMC Article
Rituximab, Omalizumab, and Dupilumab Treatment Outcomes in Bullous Pemphigoid: A Systematic Review.
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
A Systematic Review of Drug-Induced Pemphigoid.
Verheyden MJ, Bilgic A, Murrell DF
Acta Derm Venereol 2020 Aug 17;100(15):adv00224. doi: 10.2340/00015555-3457. PMID: 32176310Free 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
Nonbullous pemphigoid: A systematic review.
Lamberts A, Meijer JM, Jonkman MF
J Am Acad Dermatol 2018 May;78(5):989-995.e2. Epub 2017 Nov 1 doi: 10.1016/j.jaad.2017.10.035. PMID: 29102490

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