Desmoglein 3-Reactive B Cells “Hiding” in Pemphigus Lesions

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Paraneoplastic pemphigus herpetiformis with IgG antibodies to desmoglein 3 and without mucosal lesions.

BACKGROUND Pemphigus herpetiformis (PH) is a rare clinical entity that combines the clinical features of dermatitis herpetiformis and the immunopathologic features of pemphigus. The target antigen is usually desmoglein 1, with exceptional cases manifesting autoantibodies against desmoglein 3. More recently, it has been found that many patients with PH also demonstrate autoantibodies against des...

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Desmoglein 1 and desmoglein 3 are the target autoantigens in herpetiform pemphigus.

OBJECTIVE To determine the cell surface autoimmune target of herpetiform pemphigus (HP). DESIGN Serum samples of HP were examined by immunoblot studies with human epidermal extracts, enzyme-linked immunosorbent assay with baculovirus-expressed recombinant desmoglein (rDsg) 1 and rDsg3, and immunoadsorption assay with rDsg. PATIENTS Twenty serum samples were obtained from patients with HP wh...

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Persistence of Anti-Desmoglein 3 IgG+ B-Cell Clones in Pemphigus Patients Over Years

Pemphigus vulgaris (PV) is a prototypic tissue-specific autoantibody-mediated disease, in which anti-desmoglein 3 (Dsg3) IgG autoantibodies cause life-threatening blistering. We characterized the autoimmune B-cell response over 14 patient years in two patients with active and relapsing disease, then in one of these patients after long-term remission induced by multiple courses of rituximab (ant...

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Desmoglein 3-ELISA: a pemphigus vulgaris-specific diagnostic tool.

BACKGROUND Pemphigus vulgaris (PV) is an autoimmune-blistering disease of the skin and mucous membranes caused by autoantibodies against desmoglein 3 (Dsg3), an epidermal desmosomal adhesion protein of the cadherin family. Cloning of the Dsg3 gene and expression of the protein in a native conformation enabled the recent development of a specific and sensitive enzyme-linked immunosorbent assay (...

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IgA pemphigus showing IgA antibodies to desmoglein 1 and 3

BACKGROUND IgA pemphigus is a rare autoimmune vesiculo-pustular skin disease. Only approximately 70 cases have been reported to date. We report a case of IgA pemphigus with IgA antibodies to desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). CASE REPORT We report the case of an 60-year-old man with intraepidermal neutrophilic IgA pemphigus with IgA antibodies to Dsg1 and Dsg3. Histologic examinatio...

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ژورنال

عنوان ژورنال: Journal of Investigative Dermatology

سال: 2017

ISSN: 0022-202X

DOI: 10.1016/j.jid.2017.07.823