Oshikawa K, Sugiyama Y, Kitamura S
Department of Pulmonary Medicine, Jichi Medical School, Tochigi, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1995 Sep;33(9):1019-23.
A 64-year-old woman complained of multiple blisters in 1990. She had had a productive cough since 1975. Immunofluorescence study of a specimen obtained from a skin biopsy showed staining in a linear pattern for both IgG and C3 in the epithelial basement membrane zone (BMZ) of the dermal-epidermal junction, and a high titer of anti-BMZ antibody. These findings led to the diagnosis of bullous pemphigoid. Corticosteroid therapy was started, and resulted in some stabilization of the skin lesion. Exertional dyspnea and a productive cough developed gradually, and the patient was referred to our department in 1992. Chest X-ray film and CT findings revealed a diffuse granular shadow and bronchiectatic change, predominantly in the lower lung fields. Laboratory tests showed a high titer of cold hemagglutinin and a high level of serum immunoglobulin A. Pulmonary function tests showed a combined destructive-restrictive defect and hypoxemia (PaO2 = 58.5 Torr). From these findings diffuse panbronchiolitis was diagnosed. A low dose of erythromycin alleviated the patient's pulmonary symptoms and improved the chest radiographic findings.
一名64岁女性于1990年主诉出现多个水疱。自1975年以来她一直有咳痰性咳嗽。对取自皮肤活检的标本进行免疫荧光研究显示,在真皮 - 表皮交界处的上皮基底膜带(BMZ)中IgG和C3均呈线性染色,且抗BMZ抗体滴度很高。这些发现导致诊断为大疱性类天疱疮。开始使用皮质类固醇治疗,皮肤病变得到了一定程度的稳定。逐渐出现劳力性呼吸困难和咳痰性咳嗽,该患者于1992年转诊至我科。胸部X线片和CT检查结果显示弥漫性颗粒状阴影和支气管扩张改变,主要位于下肺野。实验室检查显示冷凝集素滴度高和血清免疫球蛋白A水平高。肺功能检查显示混合性阻塞性 - 限制性缺陷和低氧血症(动脉血氧分压 = 58.5托)。根据这些发现诊断为弥漫性泛细支气管炎。低剂量红霉素缓解了患者的肺部症状并改善了胸部X线表现。