Pulmonary involvement in Erdheim-Chester disease.
Erdheim-Chester disease is a disseminated non-Langerhans' cell histiocytosis involving multiple organs with characteristic sclerotic musculoskeletal lesions. This is the report of the case of a 53-year-old woman with extensive and progressive pulmonary disease. Computed tomography scans revealed diffuse infiltrative lung disease. Thoracoscopic lung biopsy and a biopsy of the right femur lesion were performed. The histopathology revealed that she had non-Langerhans' cell histiocytosis; Erdheim-Chester disease. The characteristic lesions of Erdheim-Chester disease, including involvement of the orbit, pericardium, periaorta, and bone were detected. This helped to further confirm that the patient had Erdheim-Chester disease with associated pulmonary involvement. As Erdheim-Chester disease is a rare non-Langerhans' cell histiocytosis that may be misdiagnosed as interstitial lung disease or other pulmonary disorders, this diagnosis should be considered in the differential diagnosis of such lung lesions.
Chung
Jae H
JH
Department of Internal Medicine, Kwandong University College of Medicine, Myungji Hospital, Koyang, Korea.
Park
Moo S
MS
Shin
Dong H
DH
Choe
Kyu O
KO
Kim
Se K
SK
Chang
Joon
J
Kim
Sung K
SK
Kim
Young S
YS
D001706:Biopsy; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005269:Femur / Q000473:pathology; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008875:Middle Aged; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed