Deciphering the Contribution of BP230 Autoantibodies in Bullous Pemphigoid.

Cole, Connor; Borradori, Luca; Amber, Kyle T (2022). Deciphering the Contribution of BP230 Autoantibodies in Bullous Pemphigoid. Antibodies, 11(3) MDPI 10.3390/antib11030044

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Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease predominantly affecting elderly patients and carries significant morbidity and mortality. Patients typically suffer from severe itch with eczematous lesions, urticarial plaques, and/or tense blisters. BP is characterized by the presence of circulating autoantibodies against two components of the hemidesmosome, BP180 and BP230. The transmembrane BP180, also known as type XVII collagen or BPAG2, represents the primary pathogenic autoantigen in BP, whereas the intracellular BP230 autoantigen is thought to play a minor role in disease pathogenesis. Although experimental data exist suggesting that anti-BP230 antibodies are secondarily formed following initial tissue damage mediated by antibodies targeting extracellular antigenic regions of BP180, there is emerging evidence that anti-BP230 IgG autoantibodies alone directly contribute to tissue damage. It has been further claimed that a subset of patients has a milder variant of BP driven solely by anti-BP230 autoantibodies. Furthermore, the presence of anti-BP230 autoantibodies might correlate with distinct clinical features. This review summarizes the current understanding of the role of BP230 and anti-BP230 antibodies in BP pathogenesis.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Borradori, Luca

Subjects:

600 Technology > 610 Medicine & health

ISSN:

2073-4468

Publisher:

MDPI

Language:

English

Submitter:

Pubmed Import

Date Deposited:

29 Jul 2022 09:18

Last Modified:

05 Dec 2022 16:22

Publisher DOI:

10.3390/antib11030044

PubMed ID:

35892704

Uncontrolled Keywords:

BP230 autoimmune blistering disease bullous pemphigoid dystonin immunobullous disease

BORIS DOI:

10.48350/171594

URI:

https://boris.unibe.ch/id/eprint/171594

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