Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Gerontology. 2011;57(3):211-6. doi: 10.1159/000315393. Epub 2010 Jul 21.
Bullous pemphigoid (BP) is an acquired autoimmune dermatosis that is often associated with various neurological diseases (ND) in the elderly. Previously, we reported that BP antigen 1 (BPAG1) of the mouse brain can be recognized by serum samples of BP patients with ND (BP+ND), indicating the possibility that BPAG1 could act as a shared autoantigen of the skin and brain. However, it is not known whether the serum of BP+ND patients could recognize BPAG1 in the human brain.
The aim of this study is to determine whether sera of BP+ND patients could recognize BP antigens in human brain tissue.
Serum samples of BP+ND patients and those of age- and sex-matched BP patients without ND, ND patients without BP, and normal controls were obtained. Serum samples were examined by immunoblotting against protein extracts of human skin and brain.
A 230-kDa protein of human epidermal extract was recognized by 16 of 22 (72.7%) serum samples of elderly BP+ND patients, whereas it was recognized by 50, 0, and 0% of serum samples of BP, ND, and controls, respectively. A 230-kDa protein of human brain extract was recognized by 12 of 22 (54.5%) serum samples of BP+ND patients, whereas it was recognized by 9.1, 9.1, and 4.5% of serum samples of BP, ND, and controls, respectively. A 180- and a 165-kDa protein could also be recognized by a proportion of serum samples of BP and BP+ND patients.
This study demonstrated that both human skin and brain contain immunogenic BPAG1 in BP+ND patients. We speculate that alterations of the central nervous system in the course of pathological changes of elderly ND patients could expose the neural isoforms of BPAG1 or other BP antigens. Autoantibodies against these antigens in the brain may cross-react with antigen in the skin, which may underlie the pathological development of BP.
大疱性类天疱疮（BP）是一种获得性自身免疫性皮肤病，常与老年人的各种神经疾病（ND）相关。此前，我们报道过，鼠脑 BP 抗原 1（BPAG1）可被伴有 ND 的 BP 患者的血清样本识别，这表明 BPAG1 可能是皮肤和脑的共同自身抗原。然而，目前尚不清楚伴有 ND 的 BP 患者的血清是否可以识别人脑中的 BPAG1。
本研究旨在确定伴有 ND 的 BP 患者的血清是否可以识别人脑中的 BP 抗原。
收集伴有 ND 的 BP 患者和年龄、性别匹配的无 ND 的 BP 患者、无 ND 的患者和正常对照者的血清样本。通过免疫印迹法检测血清样本对人皮肤和脑蛋白提取物的反应。
22 例老年伴有 ND 的 BP 患者血清样本中，有 16 例（72.7%）识别出人类表皮提取物中的 230kDa 蛋白，而 BP、ND 和对照者的血清样本分别识别出 50%、0%和 0%。22 例伴有 ND 的 BP 患者血清样本中有 12 例（54.5%）识别出人脑提取物中的 230kDa 蛋白，而 BP、ND 和对照者的血清样本分别识别出 9.1%、9.1%和 4.5%。部分 BP 和伴有 ND 的 BP 患者的血清样本还可以识别 180kDa 和 165kDa 蛋白。
本研究表明，伴有 ND 的 BP 患者的人皮肤和脑均含有免疫原性的 BPAG1。我们推测，在老年 ND 患者病理变化过程中，中枢神经系统的改变可能会暴露 BPAG1 或其他 BP 抗原的神经同工型。这些脑抗原的自身抗体可能与皮肤中的抗原发生交叉反应，这可能是 BP 病理发展的基础。
