Bullous pemphigoid: more than one disease?

Ludwig, Ralf J.

doi:10.1111/jdv.15445

Cited by 3 publications

(2 citation statements)

References 9 publications

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“…Furthermore, the injection of total IgE isolated from two BP patients sera into human skin grafted onto athymic nude mice induced erythematous plaques reminiscent of those clinically seen in BP (36). Antibodies of the IgG class reacting against BP230, combined or not with anti-BP180 antibodies, are usually found in a smaller proportion of BP patients (37). Although the actual pathogenic role of anti-BP230 autoantibodies has been initially debated (38, 39), it has been more recently well-documented (40, 41) and IgG reactivity with intracellular epitopes of BP230 has been shown to correlate to BP severity (25).…”

Section: Established Status Of Knowledge On Bullous Pemphigoid Pathogmentioning

confidence: 99%

New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update

et al. 2019

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There are several lines of evidence indicating that the physiopathological bases of bullous pemphigoid (BP), the most common subepidermal autoimmune bullous disease, are hallmarked by the production of autoantibodies directed against the hemidesmosomal anchoring proteins BP180 and BP230. In contrast to the robustness of the latter assumption, the multifaceted complexity of upstream and downstream mechanisms implied in the pathogenesis of BP remains an area of intense speculation. So far, an imbalance between T regulatory cells and autoreactive T helper (Th) cells has been regarded as the main pathogenic factor triggering the autoimmune response in BP patients. However, the contributory role of signaling pathways fostering the B cell stimulation, such as Toll-like receptor activation, as well as that of ancillary inflammatory mechanisms responsible for blister formation, such as Th17 axis stimulation and the activation of the coagulation cascade, are still a matter of debate. In the same way, the pathomechanisms implied in the loss of dermal-epidermal adhesion secondary to autoantibodies binding are not fully understood. Herein, we review in detail the current concepts and controversies on the complex pathogenesis of BP, shedding light on the most recent theories emerging from the literature.

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Section: Established Status Of Knowledge On Bullous Pemphigoid Pathogmentioning

confidence: 99%

New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update

et al. 2019

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“…IgG1 and IgG4 have been reported to be the dominant subclasses of autoantibodies in BP. IgG4 cannot trigger complement activation, so IgG1 is thought to be mainly involved in tissue damage [7]. The binding of autoantibody against hemidesmosome protein leads to complement activation and neutrophilic chemotaxis, which causes degradation of the BMZ and blister formation.…”

Section: Introductionmentioning

confidence: 99%

Factors associated with the activity and severity of bullous pemphigoid: a review

et al. 2020

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Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. It usually affects people older than 70 years of age. The two main autoantigens are BP180 and BP230, both of which are components of hemidesmosomes. Immunoglobulin (Ig)G and IgE autoantibodies to BP180 detected by the enzyme-linked immunoassay (ELISA) show close associations with the activity and severity of BP. In addition, inflammatory cells (eosinophils, neutrophils and mast cells) and cytokines (e.g. interleukins and CC chemokine ligands) play an important part in the pathogenesis, activity and severity of BP. We summarized the potential contribution of each factor postulated to be associated with the activity and severity of BP, and provide guidance for clinicians to pay timely and close attention to such parameters. This review may also promote the development of novel therapies for BP. KEY MESSAGES Bullous Pemphigoid Disease Area Index (BPDAI) is a scoring system which can reflect the extent of clinical involvement of BP patients. The titres of IgE autoantibodies and IgG autoantibodies against the NC16A domain of BP180 are closely correlated with the activity and severity of BP. Many inflammatory cells and molecules, such as eosinophils and interleukins, can also reflect the activity and severity of BP.

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Bullous pemphigoid autoantibodies

Delli¹,

Sotiriou²,

Vakirlis³

et al. 2021

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<abstract> <p>Autoimmune blistering skin disorders are rare. According to direct immunofluorescence studies, three categories are described: pemphigus group, pemphigoid group and dermatitis herpetiformis. Among these diseases, bullous pemphigoid is the most common. Patients with typical bullous pemphigoid disease are usually elderly and have many comorbidities. Considering that topical and systemic corticosteroids are the first choice therapy, these patients also have increased morbidity and risk of death. The main characteristic of bullous pemphigoid as an acquired autoimmune blistering disease is the formation of autoantibodies against hemidesmosomal antigens BP180 and BP230. Although IgG autoantibodies predominate within the plasma and skin of BP patients, some features of the disease cannot be explained solely by IgG-mediated mechanisms. Epitope spreading phenomena, immunoglobulin class switch and the relevance of IgM and IgE autoantibodies are discussed in this article.</p> </abstract>

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Bullous pemphigoid: more than one disease?

Abstract: Linked article: This is a commentary on M. Zheng et al., pp. 595–600 in this issue. To view this article visit https://doi.org/10.1111/jdv.15325.

Cited by 3 publications

References 9 publications

New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update

New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update

Factors associated with the activity and severity of bullous pemphigoid: a review

Bullous pemphigoid autoantibodies

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