PDF(Pubmed)
Abstract：
UNASSIGNED: We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
UNASSIGNED: ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared. This case demonstrates variable clinical manifestations of ECD.
UNASSIGNED: Painless bilateral proptosis with poor response to steroid treatment should prompt consideration for ECD and systemic evaluation. In addition, in the absence of typical clinical manifestations, a thorough evaluation of the biopsy can be crucial for an accurate diagnosis.
UNASSIGNED: ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared. This case demonstrates variable clinical manifestations of ECD.
UNASSIGNED: Painless bilateral proptosis with poor response to steroid treatment should prompt consideration for ECD and systemic evaluation. In addition, in the absence of typical clinical manifestations, a thorough evaluation of the biopsy can be crucial for an accurate diagnosis.
摘要：
我们报告了一名患者，该患者最初因诊断为Erdheim-Chester病（ECD）的视力丧失而就诊于眼科诊所。
■ECD是一种罕见的非朗格汉斯细胞组织细胞增生症，其特征是多系统器官受累和预后不良。在出现任何全身症状之前，我们的患者由于突出的眼眶受累而完全视力丧失。该病例表现出不同的ECD临床表现。
■对类固醇治疗反应差的无痛性双侧眼球突出应提示考虑ECD和系统评估。此外,在没有典型临床表现的情况下，对活检的全面评估对于准确诊断至关重要。
■ECD是一种罕见的非朗格汉斯细胞组织细胞增生症，其特征是多系统器官受累和预后不良。在出现任何全身症状之前，我们的患者由于突出的眼眶受累而完全视力丧失。该病例表现出不同的ECD临床表现。
■对类固醇治疗反应差的无痛性双侧眼球突出应提示考虑ECD和系统评估。此外,在没有典型临床表现的情况下，对活检的全面评估对于准确诊断至关重要。
