Two enlarged kidneys: a manifestation of Erdheim-Chester disease.
Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosis. This disorder is characterized by a bone involvement and several extraskeletal manifestations. We describe the case of a patient with a pleural and pericardial effusion leading to tamponade. Pathological examination of pericardium and mediastinal adenopathy was normal. The abdominal computed tomography scan showed two enlarged kidneys suggestive of Erdheim-Chester disease. Bone scan scintigraphy demonstrated symmetrical increased labeling of the long bones. The biopsy of perirenal soft tissue confirmed the diagnosis of Erdheim-Chester disease.
André
M
M
Department of Internal Medicine, Groupe Hospitalier Saint-Jacques, Clermont-Ferrand, France.
Delèvaux
I
I
de Fraissinette
B
B
Ponsonnaille
J
J
Costes Chalret
N
N
Wechsler
B
B
Piette
J C
JC
Aumaître
O
O
D001847:Bone Diseases / Q000209:etiology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged