IgA-mediated epidermolysis bullosa acquisita: Two cases and review of the literature

Vodegel, R.M.; Jong, M. C. J. M. de; Pas, Hendrikus; Jonkman, Marcel F.

doi:10.1067/mjd.2002.125079

Cited by 75 publications

(72 citation statements)

References 55 publications

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“…68 In rare cases, an additional staining for IgA was described. [44][45][46]74 Indirect immunofluorescence microscopy using 1 mol ⁄ L NaCl-split normal human skin as a substrate demonstrates circulating IgG autoantibodies binding to the dermal side of the artificial split in serum of EBA patients (Fig. 2b,c), which label the sublamina densa zone by indirect immunoelectron Figure 3.…”

Section: Immunofluorescence Studiesmentioning

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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Section: Immunofluorescence Studiesmentioning

confidence: 99%

Epidermolysis bullosa acquisita: What’s new?

Ishii

Hamada

Dainichi

et al. 2010

The Journal of Dermatology

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“…The clinical and histological features of their case were compatible with those of LAD but quite different from those of EBA. In addition, Vodegel et al [10] reported IgA-mediated EBA. They described that IgA-mediated EBA is clinically indistinguishable from the classic ‘lamina lucida type’ linear IgA dermatosis or from the inflammatory type of IgG-mediated EBA.…”

Section: Discussionmentioning

confidence: 99%

A Case of Mixed Bullous Disease of Epidermolysis bullosa acquisita and Linear IgA Bullous Dermatosis

Osawa¹,

Demitsu²,

Toda³

et al. 2005

Dermatology

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A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. Histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient’s IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient’s serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.

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“…Épidermolyse bulleuse acquise à IgA L'EBA à IgA touche le plus souvent l'adulte après 30 ans, même si des cas chez l'enfant ont été rapportées. Les lésions consistent en des vésicules prenant parfois une disposition annulaire, des bulles, des plaques urticariennes et des lésions en cocarde associées à un prurit intense avant ou au cours de l'éruption [13]. Les cicatrices atrophiques et les kystes milium sont rares.…”

Section: Forme Muqueuseunclassified

“…La dapsone est utilisée, après avoir exclu un déficit en G6PD, à des doses variables et est adaptée à la tolérance hématologique. Elle est efficace dans l'EBA à IgA [13] et dans le lupus vésicu-lobulleux. L'efficacité de la colchicine a été rapportée dans sept observations et est habituellement bien supportée au long cours.…”

Section: Prise En Charge Thérapeutiqueunclassified

Épidermolyse bulleuse acquise

Caux

2010

La Presse Médicale

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IgA-mediated epidermolysis bullosa acquisita: Two cases and review of the literature

Cited by 75 publications

References 55 publications

Epidermolysis bullosa acquisita: What’s new?

Epidermolysis bullosa acquisita: What’s new?

A Case of Mixed Bullous Disease of Epidermolysis bullosa acquisita and Linear IgA Bullous Dermatosis

Épidermolyse bulleuse acquise

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