title: Pulmonary involvement in Erdheim-Chester disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Kwandong University College of Medicine, Myungji Hospital, Koyang, Korea.
authors.forename: Jae H
authors.initials: JH
authors.lastname: Chung
authors.forename: Moo S
authors.initials: MS
authors.lastname: Park
authors.forename: Dong H
authors.initials: DH
authors.lastname: Shin
authors.forename: Kyu O
authors.initials: KO
authors.lastname: Choe
authors.forename: Se K
authors.initials: SK
authors.lastname: Kim
authors.forename: Joon
authors.initials: J
authors.lastname: Chang
authors.forename: Sung K
authors.initials: SK
authors.lastname: Kim
authors.forename: Young S
authors.initials: YS
authors.lastname: Kim
mesh_terms: 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