[Orbital Erdheim-Chester disease].
Erdheim-Chester disease is related to a tissue infiltration of foamy histiocytes. Results of immunoperoxydase stains for S-100 and T6 protein, the Langerhans cells antigen, is negative. It is a multisystemic disease, and it particularly involves bones and orbit. The visual prognosis is threatened, and the disease may lead to a fatal issue. Treatments have poor effects on the disease. Patients sometimes have good symptomatic response to corticotherapy. This case was revealed by headaches and diabetes insipidus. The orbital infiltration was asymptomatic.
Offret
H
H
Service d'Ophtalmologie, C.H.U. Bicêtre.
Hannouche
D
D
Frau
E
E
Doyon
D
D
Quillard
J
J
Schaison
G
G
D000328:Adult; D003937:Diagnosis, Differential; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008297:Male; D009916:Orbital Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography
Maladie d'Erdheim-Chester orbitaire.