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The bullae are formed by an immune reaction, initiated by the formation of IgG [citation needed] autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, [6] and/or type XVII collagen, also called bullous pemphigoid antigen 2, [7] which is a component of hemidesmosomes. A different form of dystonin is associated with ...
Pemphigoid is a group of rare autoimmune blistering diseases of the skin and mucous membranes.As its name indicates, pemphigoid is similar in general appearance to pemphigus, [1] however unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.
The number of target antigens varies on a case by case basis. The variability is likely what accounts for the different presentations of PNP. Through immunoprecipitation, target antigens have been found to include desmoglein-3, desmoglein-1, envoplakin, periplakin, desmoplakin 1, desmoplakin 2, and bullous pemphigoid antigen I. [citation needed]
Antibody isotype(s) and location of antibody deposition in immunofluorescence studies using salt-split skin for autoimmune bullous conditions targeting the basement membrane zone of the human integumentary system Condition Antibody isotype(s) deposited Localization of antibody with use of salt-split skin Antiepilegrin cicatricial pemphigoid ...
Gestational pemphigoid: IgG1: NC16A terminal of BPAG2 BPAG1: 180 230 Cicatricial pemphigoid: C-terminal of BPAG2: 180 Ocular cicatricial pemphigoid: Integrin beta-4 subunit: 200 Antiepiligrin cicatricial pemphigoid [nb 1] Laminin 5 (Epiligrin, Laminin 332) Linear IgA bullous dermatosis: LAD1 portion of BPAG2 Collagen type VII: 97 290/145 ...
Gastrointestinal pemphigoid: No consistent evidence of association with autoimmunity. Hypogammaglobulinemia: An immune system disorder but not autoimmune. Idiopathic giant-cell myocarditis: No consistent evidence of autoimmune cause though the disease has been found comorbid with other autoimmune diseases. [124] Idiopathic pulmonary fibrosis
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. [ 2 ] : 135 It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types: [ 3 ] : 587
Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. [3] It is one of the pemphigoid diseases that can result in scarring.