Images in Clinical Medicine

Pulmonary Langerhans'-Cell Granulomatosis (Histiocytosis X)

Raanan Arens, M.D., and Sandra S. Kramer, M.D.

N Engl J Med 2000; 342:1959June 29, 2000

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Figure 1 A previously healthy 16-year-old girl presented with a three-day history of progressive pain on the left side of her chest. A chest x-ray film showed a large left-sided pneumothorax with bilateral cystic radiolucencies, and a chest tube was inserted. A high-resolution computed tomographic scan demonstrated the pneumothorax and multiple thin-walled cysts throughout the lungs, especially in the upper lobes (Panel A). Diagnostic thoracoscopy, with the endoscope inserted in the space around the chest tube, showed multiple subpleural cysts (Panel B). A thoracoscopic wedge-biopsy specimen was obtained. Histologic evaluation demonstrated nodular aggregates of Langerhans' cells (arrows in Panel C) surrounded by dense infiltrates of eosinophils and lymphocytes (hematoxylin and eosin, ×40). Immunohistochemical staining was strongly positive for S-100 protein. Pulmonary Langerhans'-cell granulomatosis was diagnosed after no other organs were found to be involved. Treatment was begun with prednisone (2 mg per kilogram of body weight per day) and methotrexate (20 mg per square meter of body-surface area weekly). Subsequent complications included a persistent bronchopleural fistula and the recurrence of the pneumothorax on the left side and, later, on the right side, requiring pleurodesis. Thirty-eight months after the initial diagnosis, because of progressive deterioration in lung function, the patient was placed on the waiting list for double-lung transplantation.

Raanan Arens, M.D.
Sandra S. Kramer, M.D.
Children's Hospital of Philadelphia, Philadelphia, PA 19104-4399

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