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Published on: January 16, 2013
[A case of Hand-Schüller-Christian disease with pulmonary fibrosis]
H Nagai1, H Shishido, A Kurashima
1Department of Respiratory Diseases, Tokyo National Chest Hospital, Japan.
Abstract:
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.
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