Batteux F, Franck N, Jaffray P, Raulo M, Prost C, Leibowitch M, Escande J P, Weill B
Laboratoire d'Immunologie Clinique, Hôpital Cochin, Paris, France.
J Clin Immunol. 1997 May;17(3):228-33. doi: 10.1023/a:1027358528252.
Autoantibodies characteristic of autoimmune bullous skin diseases (AIBDs) can be detected by immunoblotting on epidermal, dermal, or bovine muzzle extracts. However, none of those substrates contain all the autoantigens involved in AIBDs, and the diagnosis requires the use of various substrates. Human keratinocytes were cultured under such conditions that they expressed the major autoantigens associated with AIBDs. Forty-two sera with antiepidermal antibodies were immunoblotted on the keratinocyte extract. Bands corresponding to desmoglein III, desmoglein I, BPAg2, BPAg1, and type VII collagen were found in 38 sera. Desmoplakins I and II were revealed by specific monoclonal antibodies. A review of the patients' charts showed a perfect correlation between the blots and the diagnoses of pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, cicatricial pemphigoid, and epidermolysis bullosa acquisita. Four sera revealing no band typical of AIBD were from patients with no autoimmune skin disease. Therefore, a single extract of keratinocytes can be used for the differential diagnosis of AIBDs.
自身免疫性大疱性皮肤病（AIBDs）的特征性自身抗体可通过对表皮、真皮或牛鼻提取物进行免疫印迹检测。然而，这些底物均不包含AIBDs中涉及的所有自身抗原，且诊断需要使用多种底物。在能表达与AIBDs相关的主要自身抗原的条件下培养人角质形成细胞。将42份含抗表皮抗体的血清在角质形成细胞提取物上进行免疫印迹。在38份血清中发现了与桥粒芯糖蛋白III、桥粒芯糖蛋白I、大疱性类天疱疮抗原2、大疱性类天疱疮抗原1和VII型胶原相对应的条带。桥粒斑蛋白I和II由特异性单克隆抗体显示。对患者病历的回顾显示，印迹结果与寻常型天疱疮、落叶型天疱疮、大疱性类天疱疮、瘢痕性类天疱疮和获得性大疱性表皮松解症的诊断之间存在完美的相关性。4份未显示AIBD典型条带的血清来自无自身免疫性皮肤病的患者。因此，单一的角质形成细胞提取物可用于AIBDs的鉴别诊断。
