Two enlarged kidneys: A manifestation of Erdheim-Chester disease

Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosi s. This disorder is characterized by a bone involvement and several extrask eletal manifestations. We describe the case of a patient with a pleural and pericardial effusion leading to tamponade. Pathological examination of per icardium and mediastinal adenopathy was normal. The abdominal computed tomo graphy scan showed two enlarged kidneys suggestive of Erdheim-Chester disea se. Bone scan scintigraphy demonstrated symmetrical increased labeling of t he long bones. The biopsy of perirenal soft tissue confirmed the diagnosis of Erdheim-Chester disease. Copyright (C) 2001 S. Karger AG, Basel.