Universitätsklinik für Dermatologie, Inselspital, University of Bern, 3010 Bern, Switzerland.
Br J Dermatol. 2012 Nov;167(5):1111-7. doi: 10.1111/j.1365-2133.2012.11108.x.
Prospective systematic analyses of the clinical presentation of bullous pemphigoid (BP) are lacking. Little is known about the time required for its diagnosis. Knowledge of the disease spectrum is important for diagnosis, management and inclusion of patients in therapeutic trials.
The primary aims of the study were: (i) to characterize the clinical features of BP at time of diagnosis; and (ii) to assess the diagnostic delay in BP and its impact on prognosis
All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were prospectively registered by means of a standardized data collection form.
One hundred-seventeen patients with BP were included in the study. 97cases (82.9%) had typical features with vesicles, blisters and/or erosions at time of diagnosis, while in the remaining cases (17.1%) only excoriations, eczematous and/or urticarial infiltrated lesions were observed. Head/neck as well as palmo-plantar involvement were found in up to 20% of patients, while mucosal lesions were present in 14.5% of the cases. Diagnosis was made after a mean of 6.1 months after the first symptoms. In patients, in whom the diagnostic delay was 4 months or more (defined as late diagnosis group), lesions were more often limited to one body area. The type of lesions did not affect the diagnostic delay. Diagnosis was made more rapidly in patients with limb involvement compared to those without. The calculated mortality rate in the early and late diagnosis group was 18.9% and 17.9%, respectively, without significant difference.
BP often presents with bullous lesions at time of diagnosis after a mean diagnostic delay of 6 months. Nevertheless, up to 20% of patients lack obvious blistering and postbullous erosions, mimicking thus a variety of inflammatory dermatoses. Localized disease is associated with an increased diagnostic delay, which has however no impact on prognosis.
目前缺乏对大疱性类天疱疮（BP）临床表现的前瞻性系统分析。人们对其诊断所需的时间知之甚少。了解疾病谱对于诊断、管理和将患者纳入治疗试验非常重要。
本研究的主要目的是：（i）描述 BP 诊断时的临床特征；（ii）评估 BP 的诊断延迟及其对预后的影响。
通过标准化数据收集表，前瞻性地登记了 2001 年 1 月 1 日至 2002 年 12 月 31 日期间在瑞士新诊断的所有 BP 病例。
本研究共纳入 171 例 BP 患者。97 例（82.9%）患者在诊断时具有典型表现，表现为水疱、大疱和/或糜烂，而在其余病例（17.1%）中仅观察到抓痕、湿疹样和/或荨麻疹浸润性病变。头部/颈部和手掌/足底受累在高达 20%的患者中可见，而黏膜病变在 14.5%的病例中存在。平均在出现首发症状后 6.1 个月才做出诊断。在诊断延迟为 4 个月或更长时间的患者（定义为晚期诊断组）中，病变更常局限于一个身体区域。病变类型不影响诊断延迟。有肢体受累的患者比无肢体受累的患者更快做出诊断。早期和晚期诊断组的死亡率分别为 18.9%和 17.9%，无显著差异。
BP 通常在诊断时表现为大疱性皮损，平均诊断延迟 6 个月。然而，多达 20%的患者缺乏明显的水疱和水疱后糜烂，从而模仿了各种炎症性皮肤病。局限性疾病与诊断延迟增加相关，但对预后无影响。
