Erdheim-Chester disease with chorioretinal and orbital involvement: a case report.
Arq Bras Oftalmol
; 87(5): e20220151, 2023.
Article
in En
| MEDLINE
| ID: mdl-39298729
ABSTRACT
A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Erdheim-Chester Disease
Limits:
Adult
/
Female
/
Humans
Language:
En
Journal:
Arq Bras Oftalmol
Year:
2023
Document type:
Article
Affiliation country:
Turkey
Country of publication:
Brazil