INCIDENCE AND DISTRIBUTION OF SUBEPIDERMAL AUTOIMMUNE BULLOUS SKIN DISEASES IN 3 FRENCH REGIONS
INCIDENCE AND DISTRIBUTION OF SUBEPIDERMAL AUTOIMMUNE BULLOUS SKIN DISEASES IN 3 FRENCH REGIONS
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DOI：
10.1001/archderm.131.1.48
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发表时间：
1995-01-01
影响因子：
--
通讯作者：
PROST, C
中科院分区：
文献类型：
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作者：
BERNARD, P;VAILLANT, L;PROST, C
背景与设计：根据法国亚眠、利莫日和图尔三个大学皮肤病中心在平均35个月期间收集的100例患者的前瞻性数据(包括免疫电子显微镜)，估计自身免疫性表皮下大疱性疾病的发病率和分布，对应的累积参考人口为3.55×10(6)。结果：使用这些地区的数据，我们发现自身免疫性表皮下大疱性疾病的年平均发病率为每百万人10.4例，因此，估计法国每年这些疾病的总新发病例数约为590例。根据临床和免疫电子显微镜诊断标准，确诊94例，分布如下。大疱性类天疱疮69例，瘢痕性类天疱疮12例，线状IgA皮肤病5例，妊娠疱疹4例，获得性大疱性表皮松解症2例，水泡性大疱性系统性红斑狼疮2例。我们估计大疱性类天疱疮的发病率(每年每百万人新增7例)足以将大疱性类天疱疮确定为主要的自身免疫性表皮下大疱性疾病，用于治疗试验。相反，所有其他疾病，特别是获得性大疱性表皮松解症(估计年发病率为每百万人0.17至0.26人)都非常罕见，反映出法国可用于短期临床研究的患者很少。
Background and Design: The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 X 10(6).Results: Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows. bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases.Conclusion: Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for shortterm clinical studies in France.