Immunofluorescence studies using skin sections of recessive dystrophic epidermolysis bullosa patients indicated that the antigen of anti-p200 pemphigoid is not a fragment of type VII collagen

Liu, Yuanlin; Shimizu, Hiroshi; Hashimoto, Takashi

doi:10.1016/s0923-1811(03)00092-6

Cited by 15 publications

(15 citation statements)

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“…Recently, the immunofluorescence reactivity of an anti‐p200 serum with skin of patients with recessive dystrophic epidermolysis bullosa of Hallopeau–Siemens, lacking type VII collagen, and with junctional epidermolysis bullosa of Herlitz, lacking laminin 5 was studied. In both cases, linear deposits of IgG along the DEJ were observed 15,28 . These findings demonstrate that autoantibodies in anti‐p200 pemphigoid are directed to an antigen distinct from both type VII collagen or laminin 5 15,28 (Fig.…”

Section: Immunofluorescence Studiesmentioning

confidence: 54%

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

Dilling

Rosé

Hashimoto

et al. 2006

The Journal of Dermatology

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Anti-p200 pemphigoid is a recently defined autoimmune subepidermal blistering disease characterized by circulating and tissue-bound autoantibodies to a 200-kDa protein (p200) of the dermal-epidermal junction (DEJ). This DEJ constituent is thought to be important for adhesion of basal keratinocytes to the underlying dermis. While the exact identity of p200 remains unknown, it has been demonstrated to be immunologically and biochemically distinct from all major autoantigens of the DEJ, including bullous pemphigoid antigens 180 and 230, laminin 1, 5 and 6, alpha6beta4 integrin, and type VII collagen. Clinically, most reported cases present with tense blisters as well as urticarial papules and plaques, closely resembling bullous pemphigoid. Histopathological examination of lesional skin biopsies shows subepidermal split formation and superficial inflammatory infiltrate typically dominated by neutrophils. Immunopathologically, linear deposits of immunoglobulin (Ig)G and C3 are detected along the DEJ by direct immunofluorescence microscopy of perilesional skin. Indirect immunofluorescence microscopy of patients' sera on NaCl-split human skin demonstrates circulating IgG autoantibodies labeling the dermal side of the split. By immunoblotting, these autoantibodies recognize a 200-kDa protein of human dermis. Biochemical characterization of the p200 molecule revealed a noncollagenous N-glycosylated acidic protein with an isoelectric point of approximately 5.5. We present an overview of the pathogenesis, clinical features, diagnosis and treatment of this new disease entity.

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Section: Immunofluorescence Studiesmentioning

confidence: 54%

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

Dilling

Rosé

Hashimoto

et al. 2006

The Journal of Dermatology

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“…Indeed, Morris et al [10 ]showed that the serum from a patient with anti-p200 pemphigoid did not react with the BMZ of the skin sections from RDEB patients lacking type VII collagen. On the other hand, Liu et al [11 ]showed that p200 is not a fragment of type VII collagen, because the sera from patients with anti-p200 pemphigoid did react with the BMZ of the skin sections from RDEB patients. Shimanovich et al[ 12] reported that p200 is a noncollagenous glycoprotein.…”

Section: Discussionmentioning

confidence: 99%

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

et al. 2006

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Background: Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported. Observation: A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus. A histological finding was subepidermal bullae. Direct immunofluorescence (IF) revealed a linear deposition of IgG, IgM and C3 at the epidermal basement membrane zone (BMZ). Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side. By immunoblot analysis using normal human dermal extract, the 200-kDa and 290-kDa bands were detected. Indirect IF did not show any anti-BMZ antibody activity, when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate. Conclusion: We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoantibodies to these two antigens.

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“…Further, by indirect immunofluorescence, the antilaminin 111 antibody reacted with both the dermal basement membrane zone and vessels, whereas patients' sera reacted only with the dermal basement membrane zone (1,11). Indirect immunofluorescence with skin lacking laminin 332 and type VII collagen showed that p200 does not correspond to these proteins (3,4). Biochemically, p200 is a noncollagenous N-glycosylated acidic protein (13) that is distinct from subunits of type VI collagen (13) and nidogen 2 (14).…”

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confidence: 96%

“…It is characterized by autoantibodies against a 200-kDa protein (p200) of the dermal-epidermal junction. This protein has been shown to be distinct from all other known autoantigens within the dermal-epidermal anchoring complex (3,4), including type XVII collagen (BP180) (5), bullous pemphigoid antigen 1 (BP230) (6), ␣6␤4 integrin (7), laminins 332 and 311 (8,9), and type VII collagen (10).…”

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Anti-laminin gamma-1 pemphigoid

Dainichi

Kurono

Ohyama

et al. 2009

Proc. Natl. Acad. Sci. U.S.A.

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Anti-p200 pemphigoid has been characterized by autoantibodies to an unidentified 200-kDa protein (p200) of the dermal؊epidermal junction. The objective of this study was to identify p200. We performed 2D gel electrophoresis of dermal extracts and immunoblotting with patients' sera, followed by MS analysis of a unique protein band. The protein band corresponded to laminin ␥1. Anti-laminin ␥1 mAb reacted with the anti-p200 immunoprecipitates by immunoblotting. Sera from 32 patients with anti-p200 pemphigoid showed 90% reactivity to the recombinant products of laminin ␥1. None of the healthy control sera reacted with laminin ␥1. By immunoblotting, reactivity of a patient's serum with p200 was competitively inhibited by adding anti-laminin ␥1 C-terminus mAb. Purified anti-p200 IgG also inhibited the reactivity of this mAb to dermal laminin ␥1. Most laminin ␥1-positive sera showed reactivity with recombinant laminin ␥1 C-terminal E8 fragment. Reactivity of patients' sera and purified IgG to dermal laminin ␥1 was higher than reactivity to blood vessel laminin ␥1 under reducing conditions. These results suggest that laminin ␥1 is the autoantigen for patients with anti-p200 pemphigoid. The autoantibodies may specifically recognize dermal laminin ␥1 with unique posttranslational modifications. The epitope is localized to the 246 C-terminal amino acids within the coiled-coil domain. The 9 C-terminal residues are known to be critically involved in laminin recognition by integrins.autoimmune disease ͉ basement membrane ͉ bullous pemphigoid ͉ proteomics

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Immunofluorescence studies using skin sections of recessive dystrophic epidermolysis bullosa patients indicated that the antigen of anti-p200 pemphigoid is not a fragment of type VII collagen

Cited by 15 publications

References 15 publications

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

Anti-laminin gamma-1 pemphigoid

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