Department of Dermatology, KA Rudolfstiftung, Vienna, Austria.
Br J Dermatol. 2012 Nov;167(5):1179-83. doi: 10.1111/j.1365-2133.2012.11076.x.
Anti-p200 pemphigoid is a rare subepidermal blistering disease associated with autoantibodies against a 200-kDa protein, reportedly corresponding to laminin γ1. However, direct evidence of the pathogenic potential of these antibodies has not been proven. For 5 years we have followed up a patient with anti-p200 pemphigoid. During this period she experienced a total of three generalized relapses. Quantifying our patient's autoantibody concentrations against laminin γ1 by enzyme-linked immunosorbent assay throughout the course of her disease we demonstrated a clear correlation with disease activity, thus providing the first evidence of the possible pathogenic role of antibodies against laminin γ1 in anti-p200 pemphigoid. Further analysis by Western blotting revealed the occurrence of additional autoantibodies against the α3 chain of laminin 332, 1·5 years after diagnosis, suggestive of intermolecular epitope spreading. Yet, the clinical appearance was unchanged and mucous membranes remained unaffected at any stage of the disease.
抗 p200 天疱疮是一种罕见的表皮下疱性疾病，与针对 200kDa 蛋白的自身抗体有关，据报道该蛋白对应于层粘连蛋白 γ1。然而，这些抗体的致病潜力的直接证据尚未得到证实。5 年来，我们一直在随访一位抗 p200 天疱疮患者。在此期间，她总共经历了三次全身性复发。通过酶联免疫吸附试验定量检测我们患者针对层粘连蛋白 γ1 的自身抗体浓度，我们在整个疾病过程中均显示出与疾病活动度的明确相关性，从而首次提供了针对层粘连蛋白 γ1 的抗体在抗 p200 天疱疮中可能具有致病作用的证据。进一步的 Western blot 分析显示，在诊断后 1.5 年，还发生了针对层粘连蛋白 332 的 α3 链的其他自身抗体，提示存在分子间表位扩展。然而，在疾病的任何阶段，临床表型均无变化，粘膜也未受影响。
