Joly P, Courville Ph, Lok C, Bernard Ph, Saiag Ph, Dreno B, Delaporte E, Bedane C, Picard C, Sassolas B, Plantin P, D'Incan M, Chosidow O, Pauwels C, Lambert D, Loche F, Prost C, Tancrede-Bohin E, Guillaume J C, Roujeau J C, Gilbert D, Tron F, Vaillant L
Clinique Dermatologique et INSERM U519, Institut Fédératif de Recherche Multidisciplinaire sur les Peptides, IFR23, Faculté Mixte de Médecine et de Pharmacie, Hôpital Charles-Nicolle, Rouen, France.
Dermatology. 2004;208(1):16-20. doi: 10.1159/000075040.
We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone.
To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera.
One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting.
142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5.10(-4)). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present.
These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.
我们之前提出了一套用于诊断大疱性类天疱疮（BP）的4条临床标准，包括年龄大于70岁、无萎缩性瘢痕、无黏膜受累以及颈部和头部无主要的水疱性损害。这些结果受到了质疑，因为在我们最初的研究中用作标准诊断标准的直接免疫电子显微镜检查（IEM）无法识别基底膜带的不同抗原。
以患者血清的免疫印迹分析作为主要诊断标准，重新评估这些BP临床诊断标准的有效性，以便精确识别患者血清识别的抗原。
使用真皮和表皮提取物通过免疫印迹法检测了189例各种表皮下自身免疫性水疱病（AIBD）患者的血清。对于少数血清仅识别BPAG2或免疫印迹呈阴性的患者，IEM用作补充诊断方法。
142例患者（75%）具有4条临床诊断标准中的至少3条。缺乏BP临床标准组的患者血清免疫印迹阴性的频率（34%）高于具有这些标准的患者血清（18%；p = 0.025）。具有BP临床标准组的患者血清识别BPAG1的频率（78%）高于无这些标准的患者血清（45%；p = 5.10(-4)）。相比之下，大量缺乏BP标准的患者血清识别BPAG2（71%），这与该组中大量瘢痕性类天疱疮患者的存在一致。在各种表皮下AIBD患者中，如果存在这些临床标准中的3条，则BP诊断的敏感性为86%，特异性为90%，预后阳性值极佳，超过95%。
这些结果证实了这套临床标准对BP快速诊断的价值。
