Bernard P, Enginger V, Venot J, Bedane C, Bonnetblanc J M
Service et Laboratoire de Dermatologie, CHU Dupuytren, Limoges.
Ann Dermatol Venereol. 1995;122(11-12):751-7.
Bullous pemphigoid (BP) is the most frequent autoimmune bullous skin disease and usually affects elderly patients. Despite some conflicting data, vital prognosis is poor in BP, of which mortality rate after one year of treatment varies between 10 and 40 p. 100. To date, the factors influencing this prognosis remain to be determined.
A cohort of 78 consecutive patients with BP (mean age: 80 +- 4 years) has been prospectively selected on a six-year period (1987-1992) in Limoges. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting on epidermal extracts.
The follow-up analysis (mean duration of follow-up: year of treatment (22 p. 100 in the first three months) with an actuarial survival curve inclined to be horizontal by the end of this first year of treatment. Therefore, we have studied the clinical or immunologic factors susceptible to influence the vital prognosis of BP according to this main criterion: death (or survival) at the end of the first year of treatment. None of the following factors has been found to be significantly linked to the vital prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, presence of circulating basement membrane zone autoantibodies by indirect IF. The clinical factors of bad prognosis were an altered general condition and a history of coronaropathy. The presence of circulating autoantibodies against BP180 antigen (but not the one of autoantibodies against BP230) as detected by immunoblotting on epidermal extracts was found to be significantly more frequent (50 vs 22 percent) in BP patients who died within the first year of treatment (p < 0.02).
Although modestly discriminating, the presence of circulating autoantibodies against BP180 represents the first vital prognosis factor demonstrated in BP. This result confirms the growing pathophysiologic importance of the anti-BP180 autoantibodies of which the pathogenic role has been recently formally demonstrated in BP using an animal model.
大疱性类天疱疮（BP）是最常见的自身免疫性大疱性皮肤病，通常影响老年患者。尽管存在一些相互矛盾的数据，但BP患者的重要预后较差，治疗一年后的死亡率在10%至40%之间。迄今为止，影响该预后的因素仍有待确定。
在利摩日，对1987年至1992年这六年期间连续入选的78例BP患者（平均年龄：80±4岁）进行了前瞻性研究。BP的诊断基于临床标准（使用标准化问卷）、直接免疫荧光（IF）结果（IgG和/或C3沿基底膜带的线性沉积），并通过对表皮提取物进行直接免疫电子显微镜检查和/或Western免疫印迹法进行确认。
随访分析（平均随访时间：治疗年（前三个月为22%），精算生存曲线在治疗的第一年结束时趋于水平。因此，我们根据这一主要标准研究了可能影响BP重要预后的临床或免疫因素：治疗第一年结束时的死亡（或存活）情况。未发现以下任何因素与BP的重要预后有显著关联：年龄、性别、就诊时皮肤病变范围、黏膜病变的存在、血液嗜酸性粒细胞增多、间接IF检测到的循环基底膜带自身抗体的存在。预后不良的临床因素是一般状况改变和冠心病史。通过对表皮提取物进行免疫印迹法检测发现，在治疗第一年内死亡的BP患者中，针对BP180抗原的循环自身抗体的存在（而非针对BP230的自身抗体）明显更为常见（50%对22%）（p<0.02）。
尽管区分能力有限，但针对BP180的循环自身抗体的存在是BP中首次证实的重要预后因素。这一结果证实了抗BP180自身抗体在病理生理学上日益重要，其致病作用最近已在动物模型中正式证明在BP中存在。
