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.