Hachisuka H, Kurose K, Karashima T, Mori O, Maeyama Y
Department of Dermatology, Kurume, University School of Medicine, Japan.
Arch Dermatol. 1996 Oct;132(10):1201-5.
Bullous pemphigoid is an autoimmune bullous disease with circulating anti-basement membrane zone antibodies, and it commonly affects elderly individuals; however, the reasons for the late onset of the disease are unclear.
The anti-basement membrane zone antibodies in serum samples from normal elderly subjects were compared with those in serum samples from normal young subjects.
Serum samples from 32 elderly and 28 young normal individuals and 10 patients with bullous pemphigoid were used.
Indirect immunofluorescence against guinea pig esophagus or human salt-split epidermis and immunoblotting against human and guinea pig epidermis were performed.
Serum samples from young individuals were devoid of anti-basement membrane zone antibodies against guinea pig esophagus and human salt-split epidermis. Among 32 serum samples from elderly patients, 6 cases (19%) were positive for anti-basement membrane zone antibody for guinea pig esophagus, and in those the titers were 10 in 3 cases and 40, 80, and 320 in the others. One case was positive against human split epidermis at a titer of 10. An immunoblotting analysis showed that the antigenicity of the 230-kd and 180-kd bullous pemphigoid antigen from guinea pig epidermal extract was similar to that of human epidermal extract; however, the molecular weight was slightly different. The 4 cases of elderly serum that recognized guinea pig esophagus basement membrane zone showed positivity with the 230-kd peptide in the guinea pig epidermal extract; however, they were negative with the human epidermal extracts. Direct immunofluorescence observation of these cases showed that deposition of IgG or C3 was not present in cryostat sections from flexor arm surfaces.
The serum samples from elderly subjects possessed a relatively high incidence of anti-basement membrane zone antibodies detectable with guinea pig esophagus as substrate. This observation of a specific immune defect in elderly individuals might explain why they are more susceptible to developing bullous pemphigoid.
大疱性类天疱疮是一种自身免疫性大疱性疾病,存在循环抗基底膜带抗体,常见于老年人;然而,该病发病较晚的原因尚不清楚。
将正常老年受试者血清样本中的抗基底膜带抗体与正常年轻受试者血清样本中的进行比较。
使用了32名老年和28名年轻正常个体以及10名大疱性类天疱疮患者的血清样本。
进行针对豚鼠食管或人盐裂表皮的间接免疫荧光以及针对人和豚鼠表皮的免疫印迹分析。
年轻个体的血清样本中不存在针对豚鼠食管和人盐裂表皮的抗基底膜带抗体。在32份老年患者的血清样本中,6例(19%)针对豚鼠食管的抗基底膜带抗体呈阳性,其中3例滴度为10,其他3例分别为40、80和320。1例针对人盐裂表皮呈阳性,滴度为10。免疫印迹分析表明,豚鼠表皮提取物中230-kd和180-kd大疱性类天疱疮抗原的抗原性与人表皮提取物相似;然而,分子量略有不同。4例识别豚鼠食管基底膜带的老年血清在豚鼠表皮提取物中与230-kd肽呈阳性反应;然而在人表皮提取物中呈阴性。对这些病例的直接免疫荧光观察表明,屈侧手臂表面冰冻切片中不存在IgG或C3沉积。
以豚鼠食管为底物可检测到老年受试者血清样本中抗基底膜带抗体的发生率相对较高。老年个体中这种特定免疫缺陷的观察结果可能解释了为什么他们更容易患大疱性类天疱疮。