Clinical and immunological profiles of anti-BP230-type bullous pemphigoid: Restriction of epitopes to the C-terminal domain of BP230, shown by novel ELISAs of BP230-domain specific recombinant proteins

Hayakawa, Taihei; Teye, Kwesi; Hachiya, Takahisa; Uehara, Rie; Hashiguchi, Masahiro; Kawakami, Tamihiro; Li, Xiaoguang; Tsuchisaka, Atsunari; Ohara, Koji; Sogame, Ryosuke; Koga, Hiroshi; Hamada, Takahiro; Ohata, Chika; Furumura, Minao; Ishii, Norito; Fukano, Hideo; Shimozato, Kazuo; Hashimoto, Takashi

doi:10.1684/ejd.2015.2719

Cited by 36 publications

(45 citation statements)

References 37 publications

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“…A previous study demonstrated that BP230-type BP tends to be milder than conventional BP. 13 If we consider the cases in which each BPDAI index is <10 as 'mild', then four of the six BP230-type BP cases (67%) were mild, which is consistent with a previous report, whereas none of the BP180-BP230-type BP cases were mild ( Table 1). Although IgG autoantibody subclass and complement activation have been reported as important factors in BP pathogenesis, those in BP230-type BP have been unclear.…”

Section: Discussionsupporting

confidence: 90%

“…Based on the Bullous Pemphigoid Disease Area Index (BPDAI), we evaluated Case 1 as mild and Case 2 as severe. A previous study demonstrated that BP230‐type BP tends to be milder than conventional BP . If we consider the cases in which each BPDAI index is <10 as ‘mild’, then four of the six BP230‐type BP cases (67%) were mild, which is consistent with a previous report, whereas none of the BP180‐BP230‐type BP cases were mild (Table ).…”

Section: Discussionsupporting

confidence: 89%

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Characteristics of IgG subclasses and complement deposition in BP230‐type bullous pemphigoid

Zheng

Ujiie

Iwata

et al. 2018

Acad Dermatol Venereol

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Background Bullous pemphigoid (BP) is the most common autoimmune blistering disease. BP180 is the primary autoantigen of BP, and in a portion of BP cases, BP230 is the only target of autoantibodies. Such BP is called BP230‐type BP. BP230‐type BP tends to show milder clinical phenotypes than conventional BP, but the reason is unclear. The pathogenic roles of autoantibodies and complement activation have been shown in conventional BP, but the distribution of IgG subclasses and the degree of complement deposition in BP230‐type BP remain unclear. Objective To compare the distribution of IgG subclasses and the degree of complement deposition in BP230‐type BP with those in conventional BP with autoantibodies to BP180 and BP230 (BP180‐BP230‐type BP). Methods The diagnosis of BP was confirmed by the histopathology of the lesions, the deposition of IgG and complement in the perilesional skin and the presence of circulating autoantibodies to BP180 and BP230. The disease severity was determined by bullous pemphigoid disease area index. The deposition of IgG subclasses and complement deposition were examined by direct immunofluorescence of the perilesional skin in 6 BP230‐type BP cases and 11 BP180‐BP230‐type BP cases. Results Sixty seven percent of BP230‐type BP cases show a mild clinical phenotype. All BP230‐type BP cases and 82% of BP180‐BP230‐type BP cases were found to demonstrate the clear deposition of IgG4 at the basement membrane zone of skin specimens. Notably, the deposition of IgG1 and IgG3 was faint or negative in all of the BP230‐type BP cases, whereas they were clearly detected in 91% and 64% of the BP180‐BP230‐type BP cases, respectively. The deposition of complement C3 tended to be weaker in BP230‐type BP than in BP180‐BP230‐type BP. Conclusion The mild clinical phenotype of BP230‐type BP may correlate with the weaker deposition of IgG1, IgG3 and complement in the skin lesions.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 89%

Characteristics of IgG subclasses and complement deposition in BP230‐type bullous pemphigoid

Zheng

Ujiie

Iwata

et al. 2018

Acad Dermatol Venereol

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“…In anti‐BP180 and anti‐BP230 IgG double‐positive BP patients, anti‐BP230 IgG seems to be produced through intermolecular ES. In “BP230‐type BP”, however, anti‐BP230 IgG are considered to be produced by different mechanisms . Furthermore, consistent with the BMZ structure, anti‐BP230 antibodies are also detected in other autoimmune bullous diseases, such as paraneoplastic pemphigus (PNP).…”

Section: Mechanism Of Anti‐bp230 Autoantibody Productionmentioning

confidence: 99%

“…Currently, most published work suggests that BP180‐specific immunoglobulin (Ig)G is the main pathogenic autoantibody in BP and the injection of anti‐BP180 IgG in animal models could lead to the key features of human BP. On the contrary, the function of the anti‐BP230 IgG antibody in the pathogenesis of BP is largely unknown, but several evidences suggested that anti‐BP230 IgG alone could cause the disease . These early studies investigating immune characteristics of BP focused on IgG reactivity.…”

Section: Introductionmentioning

confidence: 99%

Role of BP230 autoantibodies in bullous pemphigoid

Shih

Wang

Yuan

et al. 2020

The Journal of Dermatology

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Bullous pemphigoid (BP) is an autoimmune disease associated with subepidermal blistering due to autoantibodies directed against BP180 and BP230. BP180 is currently considered as the major pathogenic autoantigen. However, previous clinical findings suggested that anti‐BP230 autoantibodies alone can cause skin lesions in animal models and many BP patients. The characteristics of BP230 and the pathogenic roles of anti‐BP230 antibodies have been proposed. First, at the molecular level, BP230 mediates the attachment of keratin intermediate filaments to the hemidesmosomal plaque and interacts with other constituents of hemidesmosomes. Second, the presence of BP230 autoantibodies may correlate with specific clinical features of BP. The immunoglobulin (Ig)G autoantibodies from BP patients react mainly against the C‐terminus of BP230, while the IgE autoantibodies are still inconclusive. Third, in vivo, autoantibodies against BP230 involved in the disease may not only induce the inflammatory response but also impair the structural stability of hemidesmosomes. This article reviews recently published work about the role of BP230 and its antibodies, including IgG and IgE, aiming to find clues of its clinical association and lay the foundation for the research on the pathogenicity of antibodies against BP230.

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“…Notably, none of our cases showed oedematous erythema that is usually seen in typical BP. BP230‐type BP tends to exhibit phenotypes that are less severe than other forms of BP that are reactive to both BP180 and BP230 or only to BP180 . Autoantibodies to the NC16A domain of BP180 are reported to be relevant to the inflammatory phenotype of BP .…”

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confidence: 99%

A possible association between BP230-type bullous pemphigoid and dementia: a report of two cases in elderly patients

et al. 2018

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Clinical and immunological profiles of anti-BP230-type bullous pemphigoid: Restriction of epitopes to the C-terminal domain of BP230, shown by novel ELISAs of BP230-domain specific recombinant proteins

Cited by 36 publications

References 37 publications

Characteristics of IgG subclasses and complement deposition in BP230‐type bullous pemphigoid

Characteristics of IgG subclasses and complement deposition in BP230‐type bullous pemphigoid

Role of BP230 autoantibodies in bullous pemphigoid

A possible association between BP230-type bullous pemphigoid and dementia: a report of two cases in elderly patients

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