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Anti-p200 pemphigoid

MedGen UID:
1802806
•Concept ID:
C5681099
•
Disease or Syndrome
Synonym: anti-p200 pemphigoid
SNOMED CT: Anti-p200 pemphigoid (1228843008)
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:0018688
Orphanet: ORPHA454710

Definition

A rare acquired subepidermal autoimmune bullous disease with characteristics of polymorphic cutaneous lesions (blisters, urticarial lesions or scars/milia) associated with immunoglobulin G deposition in the basement membrane zone. Lesions are frequently localised on extremities, trunk, palmoplantar and cephalic areas as well as mucous membranes. The disease predominantly affects elderly people. The exact aetiology is unknown but may be related to laminin gamma-1, consistent with the identified characteristics of the p200 protein (an acidic non-collagenous N-linked glycoprotein localised within the lower lamina lucida outside of hemidesmosomes). [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnti-p200 pemphigoid

Professional guidelines

PubMed

Treatment and clinical outcomes in anti-p200 pemphigoid: a systematic review.
Laufer Britva R, Amber KT, Cohen AD, Kridin K
J Eur Acad Dermatol Venereol 2020 Mar;34(3):465-472. Epub 2019 Dec 9 doi: 10.1111/jdv.15961. PMID: 31536652
Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L
Clin Rev Allergy Immunol 2018 Feb;54(1):26-51. doi: 10.1007/s12016-017-8633-4. PMID: 28779299
Pemphigoid diseases: pathogenesis, diagnosis, and treatment.
Kasperkiewicz M, Zillikens D, Schmidt E
Autoimmunity 2012 Feb;45(1):55-70. Epub 2011 Sep 19 doi: 10.3109/08916934.2011.606447. PMID: 21923615

Recent clinical studies

Etiology

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
Annular bullous diseases.
Sussman M, Zhai L, Morquette A, Huang S, Hsu S
Clin Dermatol 2022 Sep-Oct;40(5):516-528. Epub 2022 Jan 1 doi: 10.1016/j.clindermatol.2021.12.012. PMID: 34979265
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
Anti-p200 Pemphigoid: A Systematic Review.
Kridin K, Ahmed AR
Front Immunol 2019;10:2466. Epub 2019 Oct 22 doi: 10.3389/fimmu.2019.02466. PMID: 31695695Free PMC Article
Clinical and immunological features and outcome of anti-p200 pemphigoid.
Commin MH, Schmidt E, Duvert-Lehembre S, Lasek A, Morice C, Estival JL, Debarbieux S, Rigal E, Pauwels C, De Quatrebarbes J, Roussel A, Goujon E, Stoebner PE, Jouen F, Joly P
Br J Dermatol 2016 Oct;175(4):776-81. Epub 2016 Aug 24 doi: 10.1111/bjd.14629. PMID: 27037896

Diagnosis

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
Annular bullous diseases.
Sussman M, Zhai L, Morquette A, Huang S, Hsu S
Clin Dermatol 2022 Sep-Oct;40(5):516-528. Epub 2022 Jan 1 doi: 10.1016/j.clindermatol.2021.12.012. PMID: 34979265
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
Anti-p200 pemphigoid.
Goletz S, Hashimoto T, Zillikens D, Schmidt E
J Am Acad Dermatol 2014 Jul;71(1):185-91. Epub 2014 Apr 24 doi: 10.1016/j.jaad.2014.02.036. PMID: 24767733
From anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid.
Dainichi T, Koga H, Tsuji T, Ishii N, Ohyama B, Ueda A, Natsuaki Y, Karashima T, Nakama T, Yasumoto S, Zillikens D, Hashimoto T
J Dermatol 2010 Mar;37(3):231-8. doi: 10.1111/j.1346-8138.2009.00793.x. PMID: 20507386

Therapy

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review.
Xie YH, Wang SH, Li SZ, Zuo YG
Front Immunol 2022;13:839094. Epub 2022 Mar 4 doi: 10.3389/fimmu.2022.839094. PMID: 35317170Free PMC Article
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
Clinical and immunological features and outcome of anti-p200 pemphigoid.
Commin MH, Schmidt E, Duvert-Lehembre S, Lasek A, Morice C, Estival JL, Debarbieux S, Rigal E, Pauwels C, De Quatrebarbes J, Roussel A, Goujon E, Stoebner PE, Jouen F, Joly P
Br J Dermatol 2016 Oct;175(4):776-81. Epub 2016 Aug 24 doi: 10.1111/bjd.14629. PMID: 27037896
Penicillin-induced anti-p200 pemphigoid: an unusual morphology.
Wozniak K, Kowalewski C, Hashimoto T, Ishii N, Glinska-Wielochowska M, Schwartz RA
Acta Derm Venereol 2006;86(5):443-6. doi: 10.2340/00015555-0117. PMID: 16955192
Two cases of subepidermal blistering disease with anti-p200 or 180-kD bullous pemphigoid antigen associated with psoriasis.
Yasuda H, Tomita Y, Shibaki A, Hashimoto T
Dermatology 2004;209(2):149-55. doi: 10.1159/000079602. PMID: 15316172

Prognosis

Anti-p200 Pemphigoid Mimicking Acquired Reactive Perforating Collagenosis.
Fleißner J, Ickrath K, Goebeler M, Benoit S, Kerstan A
Acta Derm Venereol 2024 Sep 9;104:adv40627. doi: 10.2340/actadv.v104.40627. PMID: 39248294Free PMC Article
State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review.
Xie YH, Wang SH, Li SZ, Zuo YG
Front Immunol 2022;13:839094. Epub 2022 Mar 4 doi: 10.3389/fimmu.2022.839094. PMID: 35317170Free PMC Article
Treatment and clinical outcomes in anti-p200 pemphigoid: a systematic review.
Laufer Britva R, Amber KT, Cohen AD, Kridin K
J Eur Acad Dermatol Venereol 2020 Mar;34(3):465-472. Epub 2019 Dec 9 doi: 10.1111/jdv.15961. PMID: 31536652
Clinical and immunological features and outcome of anti-p200 pemphigoid.
Commin MH, Schmidt E, Duvert-Lehembre S, Lasek A, Morice C, Estival JL, Debarbieux S, Rigal E, Pauwels C, De Quatrebarbes J, Roussel A, Goujon E, Stoebner PE, Jouen F, Joly P
Br J Dermatol 2016 Oct;175(4):776-81. Epub 2016 Aug 24 doi: 10.1111/bjd.14629. PMID: 27037896

Clinical prediction guides

Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN
J Am Acad Dermatol 2021 Jul;85(1):18-27. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.05.161. PMID: 33684494
Treatment and clinical outcomes in anti-p200 pemphigoid: a systematic review.
Laufer Britva R, Amber KT, Cohen AD, Kridin K
J Eur Acad Dermatol Venereol 2020 Mar;34(3):465-472. Epub 2019 Dec 9 doi: 10.1111/jdv.15961. PMID: 31536652
Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.
Kridin K
Immunol Res 2018 Feb;66(1):6-17. doi: 10.1007/s12026-017-8975-2. PMID: 29159697
Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L
Clin Rev Allergy Immunol 2018 Feb;54(1):26-51. doi: 10.1007/s12016-017-8633-4. PMID: 28779299
IgA anti-p200 pemphigoid.
Wozniak K, Hashimoto T, Fukuda S, Ohyama B, Ishii N, Koga H, Dainichi T, Kowalewski C
Arch Dermatol 2011 Nov;147(11):1306-10. doi: 10.1001/archdermatol.2011.303. PMID: 22106117

Recent systematic reviews

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review.
Xie YH, Wang SH, Li SZ, Zuo YG
Front Immunol 2022;13:839094. Epub 2022 Mar 4 doi: 10.3389/fimmu.2022.839094. PMID: 35317170Free PMC Article
Treatment and clinical outcomes in anti-p200 pemphigoid: a systematic review.
Laufer Britva R, Amber KT, Cohen AD, Kridin K
J Eur Acad Dermatol Venereol 2020 Mar;34(3):465-472. Epub 2019 Dec 9 doi: 10.1111/jdv.15961. PMID: 31536652
Anti-p200 Pemphigoid: A Systematic Review.
Kridin K, Ahmed AR
Front Immunol 2019;10:2466. Epub 2019 Oct 22 doi: 10.3389/fimmu.2019.02466. PMID: 31695695Free PMC Article

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