A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies

HASHIMOTO, T.; ISHIKO, A.; SHIMIZU, H.; TANAKA, T.; DODD, H.J.; Bhogal, B.; Black, Martin M.; NISHIKAWA, T.

doi:10.1046/j.1365-2133.1996.999721.x

Cited by 43 publications

(62 citation statements)

References 2 publications

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“…In addition to the mechanobullous classic variant, several inflammatory subtypes of EBA were described, clinically mimicking bullous pemphigoid, linear IgA disease, mucous membrane pemphigoid or Brunsting-Perry pemphigoid. [40][41][42][43][44][45][46][47][48] Although the original diagnostic criteria for EBA stated that disease onset should be in adulthood, several childhood cases have been documented. Interestingly, several previous reports described that some childhood EBA patients with reactivity to the triple-helical collagenous domain, as well as the NC1 and NC2 domains, were of the inflammatory type.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…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%

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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: Clinical Presentationmentioning

confidence: 99%

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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“…It has been known that a patient’s IgA autoantibodies react with 97- or 120-kDa protein in LAD (lamina lucida type); however, they may react with the 290-kDa type VII collagen (sublamina densa type) [9]. In the present case, circulating IgA autoantibodies against the 120-kDa linear LAD antigen LAD-1 was detected, as well as IgG antibodies to the 290-kDa EBA antigen.…”

Section: Discussionmentioning

confidence: 53%

“…Jonkman et al [2] described a case of EBA with IgG autoantibodies against type VII collagen and laminin a 3 . Hashimoto et al [9] described a patient with the sublamina densa type of LAD with IgA autoantibodies reactive with the 290-kDa type VII collagen (EBA antigen) with immunoblotting of normal human dermal extracts. The clinical and histological features of their case were compatible with those of LAD but quite different from those of 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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“…The first group of linear IgA bullous dermatosis patients, who had antibodies localized to the lamina lucida, recognized a 97-kDa peptide in human skin extracts by Western blot analysis. Immunogold-labeled antibodies and electronmicroscopy were used to demonstrate that the majority of labeling occurred in the lamina lucida beneath the hemidesmosomes (Haftek et al, 1994;Hashimoto et al, 1996;Ishiko et al, 1996). It was pointed out that although these laboratory results were very constant, the clinical picture of the patients studied varied considerably, suggesting that other factors were involved in the pathogenesis of the linear IgA bullous dermatosis (Haftek et al, 1994).…”

Section: Immunopathologymentioning

confidence: 99%

Molecular Biological Aspects of Acquired Bullous Diseases

Dabelsteen

1998

Critical Reviews in Oral Biology & Medicine

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Bullous diseases of the oral mucosa and skin were originally classified on the basis of clinical and histological criteria. The discovery of autoantibodies in some of these patients and the introduction of molecular biology have resulted in a new understanding of the pathological mechanisms of many of the bullous lesions. In this article, updated topics of the immune-mediated bullous lesions which involve oral mucosa and skin are reviewed. Pemphigus antigens, which are desmosomal-associated proteins and belong to the cadherin superfamily of cell adhesion proteins, have been isolated, and their genes have been cloned. The antigens which react with autoantibodies from patients with bullous pemphigoid, cicatricial pemphigoid, acquired epidermolysis bullosa, and linear IgA disease are all proteins of the hemidesmosome basement membrane complex. Interestingly, most of the antigens also appear to be the target for mutations seen in patients with the inherited type of epidermolysis bullosa in which bullous lesions are a prominent clinical feature.

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A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies

Cited by 43 publications

References 2 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

Molecular Biological Aspects of Acquired Bullous Diseases

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