[Erdheim-Chester disease. Apropos of a case with autopsy findings].
Erdheim-Chester's Disease is a very uncommon variety of non-Langerhans histiocytosis of unknown etiology, which characteristically affects long bones bilaterally and symmetrically in adults. It may be accompanied by visceral foci of variable localization and extension determining prognosis. Bone scintigraphy is characteristic enough to evoke the disease but histologic examination of a peripheral specimen is required to confirm the diagnosis: spumous histiocytes CD68+, PS100+/-, CD1a-. We describe a case revealed by a severe lung disease with detailed autopsy.
Ranty
M L
ML
Laboratoire d'Anatomie et de Cytologie Pathologiques, CHU Charles Nicolle, Boulevard Gambetta, 76031 Rouen.
Le Pessot
F
F
Billerey
C
C
Dominique
S
S
Métayer
J
J
D015703:Antigens, CD / Q000032:analysis; D018949:Antigens, CD1 / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D017809:Fatal Outcome; D006644:Histiocytes / Q000276:immunology / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008171:Lung Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009418:S100 Proteins / Q000032:analysis; D000097382:CD68 Molecule
D015703:Antigens, CD; D018949:Antigens, CD1; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C419393:CD1a antigen; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C110294:S100A1 protein