Anti-p200 pemphigoid (anti-laminin-γ1 pemphigoid) demonstrating pathergy
نویسندگان
چکیده
Anti-p200 pemphigoid, also called anti-laminin-γ1 pemphigoid, is a recently defined entity. First reported in 1996, the incidence is relatively rare, with approximately 70 reports in the literature. Clinical presentation is heterogeneous, but the disease most commonly mimics bullous pemphigoid with urticarial papules, plaques, or tense bullae on the trunk or extremities. Described here is a case with additional features of pathergy that have not yet been reported in the literature.
منابع مشابه
Pathogenicity of Autoantibodies in Anti-p200 Pemphigoid
Recently, the C-terminus of laminin γ1 has been identified as target antigen in anti-p200 pemphigoid and the disease was renamed as anti-laminin γ1 pemphigoid. However, the pathogenic relevance of these autoantibodies has not yet been demonstrated. Therefore, we employed an ex vivo model of autoantibody-mediated leukocyte-dependent neutrophil activation and dermal-epidermal separation (DES) usi...
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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 gamma1. Anti-laminin ga...
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INTRODUCTION Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease, first described in 1996. This disorder was initially considered as a more benign condition compared with bullous pemphigoid and epidermolysis bullosa acquisita, but in a recent series of patients, heterogeneous clinical presentations have been described, including cases with a more severe course than previou...
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