Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.
Respiration. 2013;86(4):347-51. doi: 10.1159/000351427. Epub 2013 Sep 12.
A 76-year-old man with interstitial lung disease was admitted to our institution after developing persistent dyspnea upon effort. He also had a relapse of bullous eruptions on the skin of the trunk and extremities, previously diagnosed as vesicular pemphigoid. Direct immunofluorescence of a skin biopsy specimen using fluorescence microscopy showed the linear deposition of immunglobulin A (IgA), IgG and C3 along the basement membrane. These findings indicated a definitive diagnosis of linear IgA/IgG bullous dermatosis. Chest computed tomography, bronchoalveolar lavage and transbronchial lung biopsy findings suggested nonspecific interstitial pneumonia. Direct immunofluorescence of the lung biopsy specimens using fluorescence microscopy also showed a deposition of IgA, IgG and C3 along the epithelial cell membranes and basement membranes of the bronchioles and alveoli. Lung disorders associated with linear IgA/IgG bullous dermatosis are extremely rare and, to our knowledge, this is the first report of such a case of interstitial pneumonia.
一位 76 岁男性，患有间质性肺病，因劳力性持续性呼吸困难而被收入我院。他的躯干和四肢皮肤也出现了大疱性皮疹复发，先前被诊断为疱疹样天疱疮。皮肤活检标本的直接免疫荧光通过荧光显微镜显示免疫球蛋白 A（IgA）、IgG 和 C3 沿着基底膜呈线性沉积。这些发现提示明确的线性 IgA/IgG 大疱性皮病的诊断。胸部计算机断层扫描、支气管肺泡灌洗和经支气管肺活检结果提示非特异性间质性肺炎。肺活检标本的直接免疫荧光通过荧光显微镜也显示 IgA、IgG 和 C3 沿着细支气管和肺泡的上皮细胞膜和基底膜沉积。与线性 IgA/IgG 大疱性皮病相关的肺部疾病极为罕见，据我们所知，这是首例间质性肺炎的报告。
