title: [An ophthalmologic diagnostic error leading to a rare systemic diagnosis: Erdheim-Chester disease].
abstract: BACKGROUND
  Erdheim-Chester disease is a rare systemic disease. The diagnosis is difficult due to significant clinical and morphological polymorphism. Orbital involvement is rare, but constitutes a classic means of detection.
  
  
  METHODS
  We report the case of a 60-year-old man, who consulted for evaluation of bilateral retro-orbital tumors. These tumors had been discovered on head CT two years previously during work-up of proptosis. Two biopsies were performed. The first one revealed polymorphous inflammatory tissue. The second one revealed intense granulomatous reaction, rich in non-specific foamy histiocytes. Thoracic-abdominal-pelvic CT scan detected peri-aortic and retroperitoneal infiltration. The association of these signs pointed to a diagnosis of Erdheim-Chester disease, confirmed by the re-examination of the histological samples.
  
  
  CONCLUSIONS
  Erdheim-Chester disease is a rare non-Langerhans histiocytosis with a specific tropism for perivascular and fatty connective tissue. The cause is not known. The diagnosis of this systemic disease is histological.
  
  
  CONCLUSIONS
  In the case of bilateral intra-orbital tumors, the diagnosis of Erdheim-Chester disease must be considered.
authors.affiliation: Service d'ophtalmologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France. Electronic address: marionkgilles@gmail.com.
authors.forename: M
authors.initials: M
authors.lastname: Gilles
authors.affiliation: Service de radiologie, hôpital Saint-André, 1, rue Burguet, 33075 Bordeaux, France.
authors.forename: N
authors.initials: N
authors.lastname: Alberti
authors.affiliation: Service d'anatomopathologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: C
authors.initials: C
authors.lastname: Seguy
authors.affiliation: Service d'anatomopathologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: F
authors.initials: F
authors.lastname: Leger
authors.affiliation: Service d'ophtalmologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: N
authors.initials: N
authors.lastname: Frulio
authors.affiliation: Service d'ophtalmologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: J
authors.initials: J
authors.lastname: Pechmeja
authors.affiliation: Service d'ophtalmologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: E
authors.initials: E
authors.lastname: Longueville
authors.affiliation: Service d'ophtalmologie, centre hospitalier Pellegrin, place Amélie-Raba-Léon, 33000 Bordeaux, France.
authors.forename: J-F
authors.initials: JF
authors.lastname: Korobelnik
mesh_terms: D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D003941:Diagnostic Techniques, Ophthalmological; D031249:Erdheim-Chester Disease / Q000150:complications / Q000175:diagnosis*; D005094:Exophthalmos / Q000175:diagnosis* / Q000209:etiology; D005134:Eye Neoplasms / Q000175:diagnosis*; D006801:Humans; D033162:Incidental Findings; D008297:Male; D008875:Middle Aged; D035583:Rare Diseases / Q000175:diagnosis
keywords: Erdheim-Chester disease; Histiocytose; Hystiocytosis; Maladie d’Erdheim-Chester