Interstitial pneumonia associated with linear immunoglobulin A/immunoglobulin G bullous dermatosis.
Interstitial pneumonia associated with linear immunoglobulin A/immunoglobulin G bullous dermatosis.
复制标题
与线性免疫球蛋白 A/免疫球蛋白 G 大疱性皮肤病相关的间质性肺炎。
作者：
Kakugawa T;Tomimura S;Hayashi T;Sakamoto N;Ishimatsu Y;Mukae H;Kohno S.
一名患有间质性肺病的 76 岁男性在努力后出现持续性呼吸困难后入住我们机构。他的躯干和四肢皮肤上的大疱性皮疹也复发，之前被诊断为水疱性类天疱疮。使用荧光显微镜对皮肤活检标本进行直接免疫荧光显示免疫球蛋白 A (IgA)、IgG 和 C3 沿着基底膜线性沉积。这些发现表明线性 IgA/IgG 大疱性皮肤病的明确诊断。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。使用荧光显微镜对肺活检标本进行直接免疫荧光检查也显示 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 大疱性皮肤病相关的肺部疾病极为罕见，据我们所知，这是此类间质性肺炎病例的首次报告。
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.