[Erdheim-Chester disease. The multiviceral form presenting as exophthalmos]. / Maladie d'Erdheim-Chester. Forme multiviscérale révélée par une exophtalmie.
Rev Med Interne
; 7(3): 311-7, 1986 May.
Article
em Fr
| MEDLINE
| ID: mdl-3764135
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Xantomatose
/
Exoftalmia
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
/
Male
/
Middle aged
Idioma:
Fr
Ano de publicação:
1986
Tipo de documento:
Article