Erdheim-Chester disease with chorioretinal and orbital involvement: a case report
ARQUIVOS BRASILEIROS DE OFTALMOLOGIA, cilt.87, sa.5, 2024 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 87 Sayı: 5
- Basım Tarihi: 2024
- Doi Numarası: 10.5935/0004-2749.2022-0151
- Dergi Adı: ARQUIVOS BRASILEIROS DE OFTALMOLOGIA
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
- İstanbul Üniversitesi Adresli: Evet
Özet
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 (IFN alpha-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFN alpha 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.