获得性大疱性表皮松解症的鉴定

Identification of epidermolysis bullosa acquisita

[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。

[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 inci- dence of the epidermolysis bullosa acquisita (EBA) in this group of people, and to compare standard indirect im- munofluorescence (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 37 patients with BP contained positive anti-BMZ antibodies in their sera at positive rate of 62.16%, and 14 sera were negative. 10 healthy controls were nega- tive. 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.