Bernard P, Vaillant L, Labeille B, Bedane C, Arbeille B, Denoeux J P, Lorette G, Bonnetblanc J M, Prost C
Department of Dermatology, Hôpital Dupuytren, Limoges, France.
Arch Dermatol. 1995 Jan;131(1):48-52.
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).
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.
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 short-term clinical studies in France.
根据法国亚眠、利摩日和图尔的三个大学皮肤科中心在平均35个月期间从100例患者中获得的前瞻性数据（包括免疫电子显微镜检查数据），估算自身免疫性表皮下大疱性疾病的发病率和分布情况，这些数据对应的累计参考人群为3.55×10⁶。
利用这些地区的数据，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新发病例总数约为590例。根据临床和免疫电子显微镜标准，94例患者得到了确切诊断，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大疱性皮病5例；妊娠疱疹4例；获得性大疱性表皮松解症2例；水疱大疱性系统性红斑狼疮2例。
我们的前瞻性研究首次评估了自身免疫性表皮下大疱性疾病的发病率和分布情况，该研究系统地纳入了免疫电子显微镜数据。我们估计的大疱性类天疱疮发病率（每年每百万人7例新发病例）足以将大疱性类天疱疮确定为进行治疗试验的主要自身免疫性表皮下大疱性疾病。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例）非常罕见，这反映出法国可用于短期临床研究的患者数量很少。
