Ninomiya J, Nakabayashi A, Sei Y, Takiuchi I
Department of Dermatology, Showa University Fujigaoka Hospital, Yokohama, Japan.
Dermatology. 1994;189 Suppl 1:117-9. doi: 10.1159/000246949.
A 67-year-old housewife had polymorphous eruptions with tense bullae on the extremities and upper trunk. Erosions were noted on the oral and esophageal mucosa. A skin biopsy showed subepidermal blistering with linear deposition of IgG and C3 at the basement membrane zone. In addition, intercellular deposition was observed at the lower part of the epidermis. On the basis of clinical and histological features, a diagnosis of bullous pemphigoid (BP) was made. After she was treated with prednisolone, the whole-skin lesions soon disappeared. An esophageal biopsy showed intercellular deposition of IgG and C3. Indirect immunofluorescence testing using esophageal mucosa of guinea pigs showed the same deposition at the titer of 1:20. Immunoblottings with this patient's serum revealed no reactions with either BP or pemphigus antigens. Her serum reacted strongly with a 190-kD protein, the nature of which was unknown. A treatment with ciclosporin was added to this steroid therapy, and the mucosal lesions improved gradually.
一位67岁的家庭主妇四肢和上躯干出现多形性皮疹及紧张性大疱。口腔和食管黏膜有糜烂。皮肤活检显示表皮下水疱形成,IgG和C3在基底膜带呈线性沉积。此外,在表皮下部观察到细胞间沉积。根据临床和组织学特征,诊断为大疱性类天疱疮(BP)。她接受泼尼松龙治疗后,全身皮肤病变很快消失。食管活检显示IgG和C3细胞间沉积。用豚鼠食管黏膜进行间接免疫荧光检测,在滴度为1:20时显示相同沉积。用该患者血清进行免疫印迹分析,未发现与BP或天疱疮抗原发生反应。她的血清与一种190-kD蛋白强烈反应,其性质未知。在这种类固醇治疗中加用环孢素,黏膜病变逐渐改善。