Joly P, Gilbert D, Thomine E, Delpech A, Courville P, Young P, Le Corvaisier C, Lauret P, Tron F
Dermatology Clinic, Hôpital Charles-Nicolle, Rouen, France.
Arch Dermatol. 1997 Jun;133(6):719-24.
To compare the localization of anti-basement membrane zone (BMZ) antibodies bound in vivo with the antigenic specificities of circulating anti-BMZ antibodies in patients with bullous pemphigoid (BP).
Comparison of the results of an examination of the skin specimens of the patients using direct immunoelectron microscopy and direct immunofluorescence on 1-mol/L sodium chloride-split skin with the results of an analysis of the corresponding serum samples using the immunoblot technique.
Immunodermatology department in a teaching hospital.
Thirty-six patients with typical BP and circulating anti-BMZ antibodies.
Serum samples from 22 patients with BP indicated only BP antigen 1 in the results of immunoblot analysis. Using direct immunofluorescence, an analysis of the peribullous skin samples obtained from these 22 patients showed deposits of IgG exclusively located along the epidermal side of sodium chloride-split skin; the results of direct immunoelectron microscopic examination showed deposits of IgG located on the intracellular portion of hemidesmosomes in 18 (82%) of these 22 specimens, whereas 4 biopsy specimens had linear IgG deposits located both intracellularly and extracellularly along the keratinocyte plasma membrane. The results of immunoblot analysis of the serum samples from 5 patients with BP indicated BP antigen 2 alone; the results of direct immunoelectron microscopic examination of peribullous skin samples from these 5 patients showed linear intracellular and extracellular deposits along the keratinocyte membrane, corresponding to an epidermal fluorescence labeling pattern of peribullous sodium chloride-split skin in 2 patients and a combined (dermal and epidermal) pattern in 3 patients.
The 2 different patterns of reactivity of anti-BMZ antibody deposits bound in vivo closely corresponded to the antigenic specificities indicated in the corresponding serum samples of the patients. These results are in accordance with those previously obtained in vitro and argue for identical binding profiles of circulating antibodies that are bound in vivo in BP.
比较大疱性类天疱疮（BP）患者体内结合的抗基底膜带（BMZ）抗体的定位与循环抗BMZ抗体的抗原特异性。
采用直接免疫电子显微镜和对1mol/L氯化钠分离皮肤进行直接免疫荧光检查患者皮肤标本的结果，与采用免疫印迹技术分析相应血清样本的结果进行比较。
教学医院的免疫皮肤科。
36例典型BP且有循环抗BMZ抗体的患者。
22例BP患者的血清样本在免疫印迹分析结果中仅显示BP抗原1。采用直接免疫荧光法，对从这22例患者获取的水疱周围皮肤样本进行分析，结果显示IgG沉积物仅位于氯化钠分离皮肤的表皮侧；直接免疫电子显微镜检查结果显示，在这22个标本中的18个（82%）中，IgG沉积物位于半桥粒的细胞内部分，而4个活检标本的线性IgG沉积物沿角质形成细胞质膜在细胞内和细胞外均有分布。5例BP患者的血清样本免疫印迹分析结果仅显示BP抗原2；对这5例患者水疱周围皮肤样本进行直接免疫电子显微镜检查的结果显示，沿角质形成细胞膜有线性细胞内和细胞外沉积物，2例患者对应水疱周围氯化钠分离皮肤的表皮荧光标记模式，3例患者对应联合（真皮和表皮）模式。
体内结合的抗BMZ抗体沉积物的2种不同反应模式与患者相应血清样本中显示的抗原特异性密切对应。这些结果与先前在体外获得的结果一致，并支持BP患者体内结合的循环抗体具有相同的结合谱。
