Anti-p200 pemphigoid

作者：Goletz Stephanie; Hashimoto Takashi; Zillikens Detlef; Schmidt Enno^*

来源：Journal of the American Academy of Dermatology, 2014, 71(1): 185-191.

DOI：10.1016/j.jaad.2014.02.036

• anti-p200 pemphigoid
• autoantibody
• autoantigen
• laminin gamma 1
• p200 antigen
• subepidermal blistering skin disease

Anti-p200 pemphigoid is a rare subepidermal blistering skin disease. Patients%26apos; autoantibodies label the dermal side of 1 mol/L NaCl-split human skin by indirect immunofluorescence microscopy and recognize a 200-kd protein by immunoblotting of human dermal extract. Clinically, anti-p200 pemphigoid is characterized by tense blisters and vesicles, erosions, and urticarial plaques, closely resembling bullous pemphigoid and the inflammatory variant of epidermolysis bullosa acquisita. Recently, 90% of anti-p200 pemphigoid sera were shown to recognize laminin gamma 1. The C-terminus of laminin gamma 1 was identified as an immunodominant region and in its recombinant form was used by immunoblotting and enzyme-linked immunosorbent assay for the serologic diagnosis of this disease. Subsequent ex vivo and in vivo studies were, however, unable to show pathogenic activity of antilaminin gamma 1 antibodies. Both patients%26apos; sera and sera depleted from antilaminin gamma 1 antibodies induced subepidermal splitting in an ex vivo model of autoantibody-mediated leukocyte-dependent neutrophil activation. Antilaminin gamma 1 antibodies appear to be useful biomarkers that will further facilitate the diagnosis of anti-p200 pemphigoid. The true identity of the pathogenetically relevant autoantigen of this disease, which may either be a yet unknown isoform of laminin gamma 1 or even another 200-kd protein of the dermoepidermal junction, still needs to be elucidated.

• 出版日期2014-7