Abstract：
Kimura disease (KD) is a rare, chronic, inflammatory condition, predominantly found in male patients of Asian ethnicity. It typically presents between 50-60 years of age and usually with bilateral disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) remains the main differential diagnosis, although histological analysis is essential in differentiating from other similarly presenting pathologies. In this case, we present an atypical case of unilateral orbital KD in a middle-aged, Caucasian, male gentleman and no evidence of regional lymphadenopathy along with a literature review of orbital KD and the differential diagnoses, histological features and management modalities available, adding to the sparse literature on the topic. At present, no recognised diagnostic criteria for KD are available, with histopathological analysis through incisional or excisional biopsy being the primary diagnostic method. Complete surgical excision with or without corticosteroid management remains the most common treatment modality although management is shifting to steroid-sparing immunomodulatory therapy. To the best of our knowledge, this is the first case to describe maintenance therapy of KD using mycophenolate mofetil.
摘要：
木村病（KD）是一种罕见的，慢性，炎症状况，主要见于亚裔男性患者。它通常表现在50-60岁之间，通常患有双侧疾病。血管淋巴样增生伴嗜酸性粒细胞增多(ALHE)仍是主要的鉴别诊断,尽管组织学分析对于区分其他类似的病理至关重要。在这种情况下,我们提出了一个在中年人中的单侧眼眶KD的非典型病例，高加索人,男性绅士，没有区域淋巴结病的证据以及眼眶KD和鉴别诊断的文献综述，组织学特征和可用的管理方式，增加了关于该主题的稀疏文献。目前,没有公认的KD诊断标准，通过切开或切除活检进行组织病理学分析是主要的诊断方法。尽管管理正在转向保留类固醇的免疫调节疗法，但有或没有皮质类固醇管理的完全手术切除仍然是最常见的治疗方式。据我们所知,这是第一个描述使用霉酚酸酯维持治疗KD的病例。
