[A case of Hand-Schüller-Christian disease with pulmonary fibrosis].
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.
Nagai
H
H
Department of Respiratory Diseases, Tokyo National Chest Hospital, Japan.
Shishido
H
H
Kurashima
A
A
Yoneda
R
R
Mori
M
M
Hebisawa
A
A
D000328:Adult; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D008297:Male; D011658:Pulmonary Fibrosis / Q000209:etiology*