Passive transfer of autoantibodies from a patient with mutilating epidermolysis bullosa acquisita induces specific alterations in the skin of neonatal mice

Borradori, L.

doi:10.1001/archderm.131.5.590

Cited by 25 publications

(18 citation statements)

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“…Our laboratory and others previously have attempted to transfer EBA passively into mice using concentrated IgG serum fractions and even whole blood from EBA patients without success (Shigemoto et al, 1988;Chen et al, 1992;Borradori et al, 1995). When IgG autoantibodies were injected into neonatal mice, they bound to the animal's anchoring fibrils, fixed complement, and generated an inflammatory infiltrate in the dermis, but no dermal-epidermal separation occurred.…”

Section: Figurementioning

confidence: 99%

“…First, in the study by Borradori et al (1995), the investigators used neonatal mice for IgG passive transfer with only two injections and a limited incubation time. The turnover of anchoring fibrils is thought to be low (Burgeson, 1993).…”

Section: Figurementioning

confidence: 99%

“…Consistent induction of blisters in an animal by the passive transfer of EBA IgG autoantibodies into the animal, however, has not been achieved, despite numerous attempts (Shigemoto et al, 1988;Chen et al, 1992;Borradori et al, 1995). In earlier studies, when IgG autoantibodies were injected into neonatal mice, they bound to the animal's anchoring fibrils, fixed complement, and generated an inflammatory infiltrate at the dermal-epidermal junction (DEJ), but no dermal-epidermal separation was observed (Shigemoto et al, 1988;Borradori et al, 1995). Likewise, Chen et al (1992) also injected EBA serum and whole human blood into SCID mice transplanted with human skin grafts but failed to induce blisters in either murine or grafted human skin.…”

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Evidence that Anti-Type VII Collagen Antibodies Are Pathogenic and Responsible for the Clinical, Histological, and Immunological Features of Epidermolysis Bullosa Acquisita

Woodley

Chang

Saadat

et al. 2005

Journal of Investigative Dermatology

111

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Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disease characterized by autoantibodies to type VII (anchoring fibril) collagen. Therefore, it is a prototypic autoimmune disease defined by a well-known autoantigen and autoantibody. In this study, we injected hairless immune competent mice with purified immunoglobulin G (IgG) fraction of serum from rabbits immunized with the non-collagenous amino-terminal domain (NC1) of human type VII collagen, the domain known to contain immunodominant epitopes. As a control, identical mice were injected with the IgG fraction of serum from non-immunized rabbits. Mice injected with immune IgG developed subepidermal skin blisters and erosions, IgG deposits at the epidermal-dermal junction of their skin, and circulating anti-NC1 antibodies in their serum-all features reminiscent of patients with EBA. Similar concentrations of control IgG purified from normal rabbits did not induce disease in the mice. These findings strongly suggest that autoantibodies that recognize human type VII collagen in EBA are pathogenic. This murine model, with features similar to the clinical, histological, and immunological features of EBA, will be useful for the fine dissection of immunopathogenic mechanisms in EBA and for the development of new therapeutic interventions.

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Section: Figurementioning

confidence: 99%

Section: Figurementioning

confidence: 99%

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confidence: 99%

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Evidence that Anti-Type VII Collagen Antibodies Are Pathogenic and Responsible for the Clinical, Histological, and Immunological Features of Epidermolysis Bullosa Acquisita

Woodley

Chang

Saadat

et al. 2005

Journal of Investigative Dermatology

111

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“…The probable important role of the autoantibodies in the pathogenesis of EBA has recently been illustrated experimentally. Passive transfer of purified IgG autoantibodies from a patient with severe EBA (classical type) to BALB/c mice produced histological and immunopathological alterations consistent with those seen in patients with this disease [28]. …”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

Wieme¹,

Lambert²,

Moerman

et al. 1999

Dermatology

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Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.

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“…However, serum IgE levels and the presence of antigen‐specific IgE in EBA have not been adequately studied thus far. There is only one case report showing a correlation of IgE levels with disease activity during treatment in a patient with EBA, and other reports have shown deposition of IgE on direct immunofluorescence (DIF) . In addition, anti‐BMZ IgE was reported in canine EBA .…”

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Detection of anti‐type VII collagen IgE antibodies in epidermolysis bullosa acquisita

et al. 2018

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Background Epidermolysis bullosa acquisita (EBA) is a rare pemphigoid disease involving autoantibodies to type VII collagen (COL7), a major structural component of anchoring fibrils. IgE autoantibodies to type XVII collagen (BP180) have been identified in bullous pemphigoid (BP), the prototype of pemphigoid diseases. Although the pathogenic relevance of IgG anti-COL7 has been investigated, that of IgE in EBA remains unclear. Objectives To reveal the presence and pathogenic relevance of IgE anti-COL7 in EBA. Methods We examined IgE antibodies in 109 patients with EBA by indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA).Results IIF with normal human skin revealed IgE reactivity in the basement membrane zone in 29 (26Á6%) cases. To verify whether the IgE antibodies were specific to COL7, we performed IIF with 21 clearly positive cases and the skin of a patient with dystrophic EBA, which does not involve COL7. All cases showed negative results, indicating that IgE antibodies were specific to COL7. In a modified IgG COL7 ELISA for IgE, 16 (14Á7%) cases were positive (three and 13 cases were negative and positive on IIF, respectively). We compared anti-COL7 IgG and IgE, and found a weak but significant correlation (r = 0Á459, P < 0Á001). EBA is clinically divided into a mechanobullous (MB; noninflammatory) type and an inflammatory (INF) type resembling BP. Of the IIF-positive cases, 11 of 30 (37%) had INF and nine of 48 (19%) had MB. Conclusions This study is the first to demonstrate the presence of circulating anti-COL7 IgE in patients with EBA, which may correlate with the clinical phenotype.

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Passive transfer of autoantibodies from a patient with mutilating epidermolysis bullosa acquisita induces specific alterations in the skin of neonatal mice

Cited by 25 publications

References 0 publications

Evidence that Anti-Type VII Collagen Antibodies Are Pathogenic and Responsible for the Clinical, Histological, and Immunological Features of Epidermolysis Bullosa Acquisita

Evidence that Anti-Type VII Collagen Antibodies Are Pathogenic and Responsible for the Clinical, Histological, and Immunological Features of Epidermolysis Bullosa Acquisita

Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid

Detection of anti‐type VII collagen IgE antibodies in epidermolysis bullosa acquisita

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