Zillikens D, Erhard H, Prost C, Hashimoto T, Nishikawa T, Bröcker E B
Universitäts-Hautklinik Würzburg.
Hautarzt. 1994 Mar;45(3):166-70. doi: 10.1007/s001050050057.
Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease, which can present in various clinical forms. We report on a 73-year-old male patient with the inflammatory type of EBA, which is clinically similar to bullous pemphigoid. Histological examination revealed subepidermal blister formation, and direct immunofluorescence of perilesional skin revealed a linear deposition of IgG and C3 at the basement membrane. Indirect immunofluorescence with 0.9% NaCl-separated human skin as substrate showed the presence of circulating antibodies binding to the base of the artificial blister at a titre of 1:320. Indirect immunoelectronmicroscopy revealed immune deposits localized in the area of the sublamina densa. Immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Systemic therapy with diaminodiphenyl sulphone, initially in combination with corticosteroids, resulted in complete healing of the skin lesions. With reference to this case report, we discuss the clinical pictures possible in EBA, the differential diagnosis and the treatment options.
获得性大疱性表皮松解症（EBA）是一种自身免疫性表皮下大疱病，可呈现多种临床形式。我们报告了一名73岁的男性炎性EBA患者，其临床表现与大疱性类天疱疮相似。组织学检查显示表皮下疱形成，皮损周围皮肤的直接免疫荧光显示IgG和C3在基底膜呈线性沉积。以0.9%氯化钠分离的人皮肤为底物进行间接免疫荧光显示，循环抗体以1:320的滴度结合至人工水疱底部。间接免疫电子显微镜显示免疫沉积物位于致密板下区域。真皮提取物的免疫印迹显示血清抗体与一种290 kDa的蛋白结合。用二氨基二苯砜进行全身治疗，最初联合使用皮质类固醇，使皮肤病变完全愈合。参照本病例报告，我们讨论了EBA可能出现的临床症状、鉴别诊断及治疗选择。
