The vesicular pemphigoid phenotype may be related to antibodies to a 200 kDa antigen in the lower lamina lucida

Inoh,; Nishikawa,; Hashimoto, Y.

doi:10.1046/j.1365-2133.1998.02478.x

Cited by 22 publications

(34 citation statements)

References 6 publications

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“…3). 11–21,23–25 In two cases, an additional staining for IgA was described 22,24 . Indirect immunofluorescence microscopy using NaCl‐split normal human skin as a substrate demonstrates circulating IgG autoantibodies binding to the dermal side of the artificial split (Fig.…”

Section: Immunofluorescence Studiesmentioning

confidence: 98%

“…Certain patients demonstrate a dermatitis herpetiformis‐like appearance with intensely pruritic erythematous plaques and grouped papulovesicles predominantly affecting the extensor surfaces 21 . Lesions usually heal without scarring; milia formation has been observed in some cases 19,20 . Oral and/or genital mucous membranes are affected in approximately 20% of patients with anti‐p200 pemphigoid 12,13,22,23 .…”

Section: Clinical Featuresmentioning

confidence: 99%

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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: 98%

Section: Clinical Featuresmentioning

confidence: 99%

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 described autoimmune bullous disease with autoantibodies against a 200-kD dermal antigen [1, 2, 3, 4, 5, 6, 7, 8, 9, 10]. Histologically, these cases show subepidermal blisters with neutrophil and eosinophil infiltration, and by indirect immunofluorescence (IF) there are circulating IgG autoantibodies against the dermal side of skin split with NaCl.…”

Section: Introductionmentioning

confidence: 99%

A Case of Anti-p200 Pemphigoid with Autoantibodies against both a Novel 200-kD Dermal Antigen and the 290-kD Epidermolysis bullosa acquisita Antigen

et al. 2004

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Background: Anti-p200 pemphigoid with autoantibodies against the 200-kD dermal antigen has recently been identified. Objectives: Our patient showed small and tense blisters on her face and trunk. Methods: Immunoblotting (IB), using extracts of normal human epidermis and dermal skin, and immunoelectron microscopy (IEM), using normal human skin, were performed using the patient’s serum. Results: IB analysis showed that the patient’s serum did not react with 180-kD bullous pemphigoid (BP180) or BP230 antigens; however, IgG autoantibodies in the patient’s serum reacted with a 200-kD dermal antigen as well as the 290-kD epidermolysis bullosa acquisita (EBA) antigen. IEM showed that IgG antibodies in the patient’s serum bound to the lamina lucida, as well as both the lamina densa and sublamina densa. After the treatment with prednisolone, the 290-kD protein reactivity decreased rapidly; however, the 200-kD protein band was still observed. Conclusion: We describe a rare case with immunoreactive autoantibodies against both a novel dermal 200-kD autoantigen and the 290-kD EBA antigen.

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“…Most of the patients showed clinical features resembling bullous pemphigoid [1, 2,5,6,7], dermatitis herpetiformis [8, 9] or linear immunoglobulin (Ig) A bullous dermatosis [10,11,12]. In addition, in some patients anti-p200 pemphigoid has been associated with psoriasis [1, 9, 13].…”

Section: Introductionmentioning

confidence: 99%

“…In addition, in some patients anti-p200 pemphigoid has been associated with psoriasis [1, 9, 13]. Anti-p200 pemphigoid patients usually show a good response to treatments such as oral corticosteroids [8], minocycline [14], dapsone [6, 10, 15, 16 ]and cyclosporine [1, 14]. In some cases, even corticosteroid ointment therapy has been used to control the disease [5, 17].…”

Section: Introductionmentioning

confidence: 99%

A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation

Goto-Ohguchi

Nishie²,

Akiyama³

et al. 2008

Dermatology

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Anti-p200 pemphigoid is a recently described autoimmune blistering skin disease that is characterized by the presence of autoantibodies against an unidentified 200-kDa dermal autoantigen. Most of the previous cases have been successfully treated using mild-to-moderate immunosuppressive therapies, which resulted in a good prognosis. We report here a severe and refractory case of anti-p200 pemphigoid that developed in a 53-year-old woman, in which blisters led to multiple skin ulcers, followed by severe scar formation. In the present case, methylprednisolone pulse therapy was effective enough to reduce the disease activity.

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The vesicular pemphigoid phenotype may be related to antibodies to a 200 kDa antigen in the lower lamina lucida

Cited by 22 publications

References 6 publications

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

A Case of Anti-p200 Pemphigoid with Autoantibodies against both a Novel 200-kD Dermal Antigen and the 290-kD Epidermolysis bullosa acquisita Antigen

A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation

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