Structural proteins of the dermal‐epidermal junction targeted by autoantibodies in pemphigoid diseases

Goletz, Stephanie; Zillikens, Detlef; Schmidt, Enno

doi:10.1111/exd.13446

Cited by 83 publications

(80 citation statements)

References 157 publications

(160 reference statements)

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“…The hemidesmosomal antigens BP 230 (BP Antigen 1) and BP180 (BP antigen 2 or collagen XVII) are the main targets in BP . Autoimmunity and loss of tolerance results in IgGs targeting the non collagenous region (NC16A) of the BP 180 ectodomain .…”

Section: Bullous Pemphigoidmentioning

confidence: 99%

Review of autoimmune blistering diseases: the Pemphigoid diseases

Daniel

Murrell

2019

Acad Dermatol Venereol

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Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub‐epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease.

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Section: Bullous Pemphigoidmentioning

confidence: 99%

Review of autoimmune blistering diseases: the Pemphigoid diseases

Daniel

Murrell

2019

Acad Dermatol Venereol

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“…On the one hand, through its C‐terminal tail containing PRD, BP230 specifically interacts with the IF network formed by keratins 5 and 14 and thereby connecting the cytokeratin network of basal keratinocytes to the extracellular matrix. On the other hand, through its N‐terminal domains, BP230 binds to the β4 subunit of α6β4 integrin and BP180, which are components of the anchoring filaments of the extracellular matrix . Additionally, plectin is associated with the keratin IF and the cell surface receptor α6β4 integrin (Fig.…”

Section: Physiological Function Of Bp230mentioning

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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“…In pemphigoid diseases, autoantibodies attach along the dermal‐epidermal junction and induce subepidermal blistering, which manifests clinically as tense blisters. Depending on the target antigen and the clinical presentation, pemphigoid diseases are subdivided into bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), anti‐p200/laminin‐γ1 pemphigoid, pemphigoid gestationis (PG), linear IgA disease, and epidermolysis bullosa acquisita (Table , Figure ). The incidence of AIBDs varies substantially, depending on ethnicity and geographical region .…”

Section: Introductionmentioning

confidence: 99%

Diagnosis of autoimmune bullous diseases

Beek

Zillikens

Schmidt

2018

J Deutsche Derma Gesell

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Autoimmune bullous disorders (AIBDs) are a heterogeneous group of rare diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. AIBDs can be categorized into two groups: pemphigus diseases, characterized by intraepidermal blistering and autoantibodies against desmosomal proteins such as desmoglein (Dsg) 1, Dsg3, members of the plakin family, and subepidermal AIBDs, comprised of pemphigoid diseases and dermatitis herpetiformis. Autoantibodies in dermatitis herpetiformis target transglutaminases 2 and 3, while in pemphigoid disease, autoantibodies are directed against structural proteins of the dermal-epidermal junction. Although analysis of a perilesional biopsy with direct immunofluorescence (IF) microscopy is still the diagnostic gold standard, several assays have become widely available that allow serological diagnosis in the majority of patients. Standard serological diagnosis includes indirect IF on monkey esophagus and salt-split human skin. Assays to further characterize autoantibody specificity include ELISA systems based on recombinant forms of the immunodominant regions of the target antigens as well as multivariant indirect IF microscopy tests with several miniature substrates. These serological assays are complemented by various in-house assays using immunoblotting and ELISA, which are only available in specialized laboratories. Here we review new developments in the diagnosis of AIBDs and describe state-of-the-art diagnostic procedures for this group of diseases.

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Structural proteins of the dermal‐epidermal junction targeted by autoantibodies in pemphigoid diseases

Cited by 83 publications

References 157 publications

Review of autoimmune blistering diseases: the Pemphigoid diseases

Review of autoimmune blistering diseases: the Pemphigoid diseases

Role of BP230 autoantibodies in bullous pemphigoid

Diagnosis of autoimmune bullous diseases

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