获得性大疱性表皮松解症的鉴定

Identification of epidermolysis bullosa acquisita

关键词:  盐裂皮肤  间接免疫荧光  表皮下大疱病  基底膜带

Key words:  salt-split skin  indirect immunofluorescence  subepidermal bullous disease and basement membrane zone

摘要:

［目的］ 对经过临床表现、病理、直接免疫荧光等常规手段诊断为大疱性类天疱疮（BP）的患者血清进行回顾性研究，以确定获得性大疱性表皮松解症（EBA）在该组人群中的发生率，并比较常规间接免疫荧光（IIF）技术和盐裂皮肤IIF技术在检测BP患者血清中抗基底膜带（BMZ）抗体的敏感性。［方法］ 分别应用常规IIF技术和盐裂皮肤IIF技术对37例BP患者及10例正常对照血清中抗BMZ抗体进行检测。［结果］ 常规IIF示37例BP患者中23例血清抗BMZ抗体阳性，阳性率为62.16%，10例正常对照血清阴性；盐裂皮肤IIF示37例BP患者中35例血清抗BMZ抗体阳性，阳性率为94.59%，其中31例为表皮侧IgG沉积、1例为真皮侧沉积、3例为双侧沉积，10例正常对照血清阴性。盐裂皮肤IIF检测BP患者血清中抗BMZ抗体的阳性率与常规IIF相比明显升高，具有显著性差异（p=0.001<0.01）。 ［结论］ 37例BP患者中检出1例EBA（盐裂皮肤IIF示真皮侧沉积），EBA在经过常规方法诊断为BP的患者中的发生率为2.7%（1/37），检测BP患者血清抗BMZ抗体时盐裂皮肤IIF技术比常规IIF技术敏感。

Abstract:

［Objective］ To study retrospectively the serum samples of the patients routinely diagnosed as having bullous pemphigoid (BP) by the clinical features, pathology and direct immunofluorescence to obtain the incidence of the epidermolysis bullosa acquisita (EBA) in this group of people, and to compare standard indirect immunofluorescence (IIF) and salt-split skin IIF in sensibility while testing the anti-basement membrane zone (BMZ) antibodies of the serum samples of the patients with BP. ［Methods］ The anti-BMZ antibodies of serum samples of 37 patients with BP and 10 healthy controls were tested by standard indirect IIF and salt-split skin IIF, respectively. ［Results］ Standard IIF showed that 23 of 37patients with BP contained positive anti-BMZ antibodies in their sera at positive rate of 62.16%, and 14 sera were egative. 10 healthy controls were negative. Salt-split skin IIF showed that 35 of 37 patients with BP contained positive anti-BMZ antibodies in their sera at positive rate of 94.59 %, 31 of them were with IgG stained epidermal side, 1 of them was with IgG stained dermal side, 3 of them were with IgG stained both sides, and 2 sera were negative. 10 healthy controls were negative. The positive rate of salt-split skin IIF was significantly higher than that of Standard IIF (P=0.001<0.01) ［Conclusions］ Recording to the anthors only 1 of 37 patients was tested the EBA (salt-split skin IIF showed IgG stained dermal side), that is, 2.7 % of the patients with BP by standard diagnoses had EBA, and salt-split skin IIF was higher in sensibility than standard IIF while testing anti-BMZ antibodies of serum samples of patients with BP.