[Erdheim-Chester disease. The multiviceral form presenting as exophthalmos].
Erdheim-Chester disease is a rare visceral xantho-granulomatosis, the 17th case of which is reported here. The initial symptom, bilateral exophthalmos, was uncommon. The picture was completed by a retroperitoneal xanthogranuloma and by bilateral and symmetrical osteosclerosis of the long bones. The lack of X-bodies at electron microscopy differentiated the disease from Hand-Schüller-Christian disease. The patient's condition improved with chemotherapy (vinblastine and doxorubicin) combined with corticosteroid therapy. After a 5-month remission period, he died of an intercurrent infection. No autopsy was performed.
D003937:Diagnosis, Differential; D005094:Exophthalmos / Q000209:etiology*; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010026:Osteosclerosis / Q000150:complications / Q000473:pathology; D012187:Retroperitoneal Space; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014973:Xanthomatosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology
Maladie d'Erdheim-Chester. Forme multiviscérale révélée par une exophtalmie.