Dermatology Department, Hospital Clínic, Villarroel 170, 08036, Barcelona, Spain.
Br J Dermatol. 2014 Nov;171(5):1022-30. doi: 10.1111/bjd.13144. Epub 2014 Oct 15.
Epidermolysis bullosa acquisita is an exceedingly rare subepidermal blistering disease caused by antibodies against type VII collagen. Studies summarizing the clinical and immunological features of this disease in large series of patients are scarce.
To analyse the clinical and immunopathological characteristics, treatment responses and outcomes of 12 patients with epidermolysis bullosa acquisita from four tertiary hospitals in Spain.
An extensive retrospective review of clinical charts.
The mean age of onset was 48 years and the mean delay to diagnosis was 20·75 months. The classical phenotype occurred in 42% of cases, inflammatory in 42% and mixed in 17%. Mucosal involvement was present in 75%. Linear IgG deposition along the basement membrane zone was consistently present on direct immunofluorescence examination. Indirect immunofluorescence study was positive in 67% of the cases. Frequently associated diseases were neoplasms (25%), inflammatory bowel disease (25%), hepatitis C virus infection (17%) and thyroid dysfunction (17%). Therapeutic responses were variable.
The prevalence of neoplasms was similar to that seen in inflammatory bowel disease. Multicentric prospective studies including larger numbers of patients are required for a better knowledge and management of this disease.
获得性大疱性表皮松解症是一种极为罕见的表皮下水疱性疾病，由针对 VII 型胶原的抗体引起。总结此类疾病在大量患者中临床和免疫学特征的研究较为匮乏。
分析来自西班牙 4 家三级医院的 12 例获得性大疱性表皮松解症患者的临床和免疫病理特征、治疗反应和结局。
对临床病历进行广泛的回顾性分析。
发病的平均年龄为 48 岁，平均诊断延误时间为 20.75 个月。经典表型占 42%，炎症表型占 42%，混合表型占 17%。75%的患者存在黏膜受累。直接免疫荧光检查始终存在线性 IgG 沿基底膜带沉积。间接免疫荧光研究在 67%的病例中呈阳性。常合并的疾病有肿瘤（25%）、炎症性肠病（25%）、丙型肝炎病毒感染（17%）和甲状腺功能障碍（17%）。治疗反应各异。
肿瘤的患病率与炎症性肠病相似。需要开展包括更多患者的多中心前瞻性研究，以更好地了解和管理此类疾病。
