Bullous pemphigoid in young patients:: a retrospective study of 74 cases
Bullous pemphigoid in young patients:: a retrospective study of 74 cases
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DOI：
10.1016/s0151-9638(05)79220-6
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发表时间：
2005-02-01
影响因子：
0.9
通讯作者：
Richard, MA
中科院分区：
文献类型：
--
作者：
Bourdon-Lanoy, E;Roujeau, JC;Richard, MA
导言。大疱性类天疱疮通常影响老年人。仅有少数65岁以下人群中的孤立病例被报道。描述年龄在60岁以下的大疱性类天疱疮患者的临床和生物学特征，将其与老年人的常见特征进行比较，并寻找潜在的病理联系。回顾性、全国性、多中心研究。采用标准化问卷调查，记录患者的临床、生物学和组织学特征以及治疗方法和相关病理改变。对1970年6月至2002年3月收治的74例大疱性类天疱疮进行分析。平均发病年龄为46岁+/-11.6岁。对42例患者进行了分离皮肤的间接免疫荧光和/或免疫电子显微镜和/或免疫印迹的进一步探索(56.8，第100页)。在这一受限人群中发现的临床特征与在其他32例中发现的相似。与老年人大疱性类天疱疮的通常数据相比，我们观察到广泛性疾病的比例更高(75页。100)，头颈部受累更频繁(39.2页。100)，以及抗BP180自身抗体的过度表达(48页。100)。肿瘤报告7例(9.5 p.100)，基底膜病变18例(24.3 p.100)(银屑病6例，特应性皮炎6例，苔藓6例)，神经系统疾病13例，其中4例卧床不起。长期药物治疗46例(62.2 p.100)，平均2.12+/-2.43，PUVA治疗4例，放射治疗2例。我们的结果表明，年轻人中的大疱性类天疱疮比老年人中常见的类天疱疮更严重、更活跃。这种特殊的形式可能是抗BP180自身抗体高表达的结果，抗BP180自身抗体被认为是这种疾病预后不良的标志。我们还发现了高频率的病理联系和物理治疗，所有这些都对基底膜区域的破坏负责，这可能涉及针对半桥粒组分的自动免疫。
Introduction. Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported.Objectives. Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations.Patients and methods. Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies.Results. Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy.Discussion. Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.