Bullöse Autoimmundermatosen (I): Pathogenese und Diagnostik

Sitaru, Cassian; Goebeler, Matthias; Zillikens, Detlef

doi:10.1046/j.1439-0353.2004.03642.x

Cited by 16 publications

(32 citation statements)

References 8 publications

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“…Autoantibodies in bullous pemphigoid are directed against two hemidesmosomal antigens, BP230 and BP180/type XVII collagen [87]. Pemphigoid gestationis, also referred to as herpes gestationis, is a subepidermal blistering disease associated with pregnancy and characterized by linear deposition of C3 and, to a lesser extent of IgG at the dermal–epidermal junction, as detected by immunofluorescence microscopy [62, 66]. The autoimmune response in bullous pemphigoid and pemphigoid gestationis is mainly directed against epitopes clustered within the immunodominant 16th non-collagenous (NC16) A region of type XVII collagen [48, 65, 88].…”

Section: Subepidermal Autoimmune Blistering Diseasesmentioning

confidence: 99%

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The relevance of the IgG subclass of autoantibodies for blister induction in autoimmune bullous skin diseases

2007

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Autoimmune bullous skin diseases are characterized by autoantibodies and T cells speciWc to structural proteins maintaining cell-cell and cellmatrix adhesion in the skin. Existing clinical and experimental evidence generally supports a pathogenic role of autoantibodies for blister formation. These autoantibodies belong to several IgG subclasses, which associate with diVerent functional properties and may thus determine the pathogenic potential of IgG antibodies. In pemphigus diseases, binding of IgG to keratinocytes is suYcient to cause intraepidermal blisters without engaging innate immune eVectors and IgG4 autoantibodies seem to mainly mediate acantholysis. In contrast, in most subepidermal autoimmune blistering diseases, complement activation and recruitment and activation of leukocytes by autoantibodies are required for blister induction. In these conditions, tissue damage is thought to be mainly mediated by IgG1, but not IgG4 autoantibodies. This review summarizes the current knowledge on the pathogenic relevance of the IgG subclass of autoantibodies for blister formation. Characterization of the pathogenically relevant subclass(es) of autoantibodies not only provides mechanistic insights, but should greatly facilitate the development of improved therapeutic modalities of autoimmune blistering diseases.

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Section: Subepidermal Autoimmune Blistering Diseasesmentioning

confidence: 99%

“…Autoimmune blistering diseases are associated with an autoimmune response directed to structural proteins mediating cell–cell and cell–matrix adhesion in the skin [62, 66]. Both autoantibodies and autoreactive T cells have been found in patients with these organ-specific autoimmune diseases.…”

Section: Introductionmentioning

confidence: 99%

The relevance of the IgG subclass of autoantibodies for blister induction in autoimmune bullous skin diseases

2007

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“…pemphigoid diseases and epidermolysis bullosa acquisita [EBA]). [3][4][5] The term ''epidermolysis bullosa acquisita'' was proposed as a descriptive clinical diagnosis for patients with adult onset and features resemble those of hereditary dystrophic epidermolysis bullosa were reported by Elliott in 1904. 6 In 1971, Roenigk et al…”

Section: Introductionmentioning

confidence: 99%

Epidermolysis bullosa acquisita: What’s new?

Ishii

Hamada

Dainichi

et al. 2010

The Journal of Dermatology

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Type VII collagen is an adhesion molecule of the extracellular matrix in epithelial basement membranes, and the main constituent of anchoring fibrils at the dermal-epidermal junction (DEJ). Autoimmunity against this protein is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). EBA is a rare acquired, heterogeneous, chronic blistering disease of skin disease of skin and mucous membranes characterized by subepidermal blisters and tissue-bound as well as circulating autoantibodies to the DEJ. EBA has several distinct clinical presentations with other subepidermal bullous diseases, such as mainly dystrophic epidermolysis bullosa or bullous pemphigoid. The circulating immunoglobulin G autoantibodies for EBA react with a 290-kDa dermal protein, type VII collagen, as detected by immunoblot analysis using dermal extracts. The pathogenicity of these autoantibodies has been demonstrated by experimental animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA cases often require high doses of systemic corticosteroids and a variety of immunosuppressants. Although treatment for EBA is frequently difficult and unsatisfactory, some therapeutic success has been reported with colchicine, dapsone, infliximab and i.v. immunoglobulin. In this review, we will focus on recent progress in our understanding of the clinical manifestations, the etiopathogenesis as well as the management of EBA.

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“…Patients with ABSDs develop autoantibodies reactive with the epidermal keratinocyte cell surfaces or the epidermal basement membrane zone which, in turn, induce separation between epidermal keratinocytes or at the dermal-epidermal junction [1,2]. Based on histopathological, immunological, and clinical criteria, autoimmune bullous diseases are classified into two major groups associated with autoantibodies to desmosomal (pemphigus group) or hemidesmosomal proteins (subepidermal blistering diseases, e.g., pemphigoid diseases and epidermolysis bullosa acquisita (EBA)) [3][4][5][6]. Patients suffering from ABSDs are treated with high doses of systemic corticosteroids mostly in combination with other immunosuppressants [7,8].…”

Section: Introductionmentioning

confidence: 99%

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

Ishii

Hashimoto

Zillikens

et al. 2009

Clinic Rev Allerg Immunol

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Treatment of autoimmune bullous skin diseases can often be challenging and primarily consists of systemic corticosteroids and a variety of immunosuppressants. Current treatment strategies are effective in most cases but hampered by the side effects of long-term immunosuppressive treatment. Intravenous immunoglobulin (IVIG) is one potential promising therapy for patients with autoimmune bullous skin diseases, and evidence of its effectiveness and safety is increasing. A number of autoimmune bullous skin diseases have been identified in which IVIG treatment may be beneficial. However, experience with IVIG in patients with autoimmune skin blistering disease is limited, where it is recommended for patients not responding to conventional therapy. The mode of action of IVIG in autoimmune diseases, including bullous diseases is far from being completely understood. We here summarize the clinical evidence supporting the notion, that IVIG is a promising therapeutic agent for the treatment of patients with autoimmune bullous skin disease. In addition, we review the proposed modes of action. In the future, randomized controlled trials are necessary to better determine the efficacy and adverse effects of IVIG in the treatment of autoimmune bullous skin diseases. In addition, insights into IVIG's mode of action might enable us to develop novel therapeutics to overcome the current shortage of IVIG.

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Bullöse Autoimmundermatosen (I): Pathogenese und Diagnostik

Cited by 16 publications

References 8 publications

The relevance of the IgG subclass of autoantibodies for blister induction in autoimmune bullous skin diseases

The relevance of the IgG subclass of autoantibodies for blister induction in autoimmune bullous skin diseases

Epidermolysis bullosa acquisita: What’s new?

High-Dose Intravenous Immunoglobulin (IVIG) Therapy in Autoimmune Skin Blistering Diseases

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