[Erdheim-Chester disease presenting with pulmonary lesion].
A 49-year-old man first visited our hospital in 1991 for further examination of abnormal pulmonary shadows. A chest radiograph and computed tomographic (CT) scan showed diffuse reticular shadows in both lung fields. The findings from a transbronchial lung biopsy specimen were not conclusive. Although there was little change in the abnormal pulmonary shadows, the patient's lung functions gradually deteriorated, indicating an obstructive defect. The patient was admitted in 1998 with the chief complaint of increasing dyspnea on exertion. A thoracoscopic lung biopsy specimen revealed proliferation of histiocytes with fibrosis in the pleura and perivascular interstitium. Immunohistochemically, the histiocytic cells were CD68-positive, alpha 1-antichymotripsin-positive, S100 protein-negative, and CD1a-negative. A bone scintigram and magnetic resonance images showed symmetrical diametaphyseal bone lesions in the distal femurs and the proximal tibiae; however, the epiphyses were spared. These findings were consistent with Erdheim-Chester disease. This is the first reported case of Erdheim-Chester disease with pulmonary involvement in Japan.
Department of Respiratory Medicine, Matsue Hospital, Shimane, Japan.
H
H
Nakano
S
S
Yano
K
K
Kobayashi
Y
Y
Kawasaki
M
M
Mikami
S
S
Shishido
M
M
Fukuda
Y
Y
Kawabata
D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology*; D000514:alpha 1-Antichymotrypsin / Q000032:analysis; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D015415:Biomarkers; D000514:alpha 1-Antichymotrypsin; C067980:CD68 antigen, human; D000097382:CD68 Molecule