Pulmonary Erdheim-Chester disease: A response to predonisolone

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhan’s cell histiocytosis of unknown origin, involving multiple organs. The patient with ECD described here is a 38-year-old man who was admitted to the hospital with dyspnea on exertion. His chest radiograph revealed a diffuse reticulonodular shadow. After the video-assisted thoracoscopic surgery was performed, he was diagnosed as having ECD. A brown eruption on his left temple, when tested by skin biopsy, proved to be ECD. No lesions other than these on the lung and skin were identified, and oral administration of predonisolone successfully treated both of them. Although recovery has followed the administration of predonisolone and chemotherapy for several patients with pulmonary ECD, this is the first report that predonisolone alone provided clinical and objective recovery from pulmonary ECD. This outcome indicates that, of all the many treatments tried for ECD, steroids may become the first-line therapy for pulmonary involvement.