Delaporte E, Piette F, Janin A, Cozzani E, Joly P, Thomine E, Nicolas J F, Bergoend H
Service de Dermatologie A, CHRU, Lille.
Ann Dermatol Venereol. 1995;122(1-2):19-22.
Subepidermal autoimmune bullous dermatoses form a clinical entity for which there is not always an individualized clinical and pathological description.
A patient presented with bullous skin disease of atypical nature. There was an almost total desepidermization of the legs, vast areas of erosion on the trunk and arms with a Nikolski sign in an area of healthy skin, buccal involvement, multiple milium cysts and ungueal dystrophies with nail loss.
This clinical presentation in this patient suggested acquired bullous epidermolysis. However, according to the recently defined clinical criteria for pemphigoid, the probability of correct diagnosis of pemphigoid was greater than 95 p. 100 since nearly three fourths of the major criteria were present. This diagnosis was confirmed by reference techniques (electron microscopy, indirect electron immunomicroscopy and immunoblotting). Thus, bullous autoimmune diseases of the dermoepidermal junction can be reliably differentiated on the bases of the clinical features, together with direct and indirect immunofluorescence on salt-split skin.
表皮下自身免疫性大疱性皮肤病构成一种临床实体，对此并非总能有个体化的临床和病理描述。
一名患者出现非典型性大疱性皮肤病。腿部几乎完全表皮剥脱，躯干和手臂有大片糜烂，在健康皮肤区域有尼氏征，口腔受累，有多个粟丘疹囊肿以及甲营养不良伴指甲脱落。
该患者的临床表现提示获得性大疱性表皮松解症。然而，根据最近定义的类天疱疮临床标准，类天疱疮正确诊断的概率大于95%，因为几乎四分之三的主要标准都存在。通过参考技术（电子显微镜、间接电子免疫显微镜和免疫印迹）证实了这一诊断。因此，基于临床特征以及盐裂皮肤的直接和间接免疫荧光，真皮表皮交界处的大疱性自身免疫性疾病能够得到可靠的鉴别。
