Nagai H, Shishido H, Kurashima A, Yoneda R, Mori M, Hebisawa A
Department of Respiratory Diseases, Tokyo National Chest Hospital, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1992 Jun;30(6):1141-5.
A 42-year-old man was admitted with dyspnea and abnormal shadows on chest roentgenogram, which showed bilateral reticulonodular shadows, multiple pulmonary cysts, and pleural effusion. A month after admission, he developed severe respiratory failure and chest X-ray revealed exacerbation of reticulonodular shadows. Steroid pulse therapy improved the symptoms and chest X-ray. He had atrophy of the mandible. Bone scintigram revealed multiple hot lesions. Bone biopsy of the right tibia showed lipogranuloma, and the diagnosis of Hand-Schüller-Christian disease (HSC) was made. The percentage of OKT6-positive cells (Langerhans cells) found by immunofluorescence was 16.1% of all cells in bronchoalveolar lavage fluid, indicating pulmonary involvement due to HSC. This is a rare case of Hand-Schüller-Christian disease with pulmonary fibrosis.
一名42岁男性因呼吸困难及胸部X线片出现异常阴影入院，胸部X线片显示双侧网状结节状阴影、多发肺囊肿及胸腔积液。入院1个月后，他出现严重呼吸衰竭，胸部X线显示网状结节状阴影加重。类固醇冲击疗法使症状及胸部X线表现有所改善。他存在下颌骨萎缩。骨闪烁显像显示多个热区。右胫骨骨活检显示脂肪肉芽肿，诊断为汉-许-克病（HSC）。通过免疫荧光法检测发现支气管肺泡灌洗液中OKT6阳性细胞（朗格汉斯细胞）占所有细胞的16.1%，提示HSC累及肺部。这是一例罕见的伴有肺纤维化的汉-许-克病。
