Youssef Alexey, Ali Rahaf, Ali Kinan, AlShehabi Zuheir
Faculty of Medicine, Tishreen University, Lattakia, Syria.
Cardiology Department, Tishreen University hospital, Lattakia, Syria.
Oxf Med Case Reports. 2017 Jun 15;2017(6):omx024. doi: 10.1093/omcr/omx024. eCollection 2017 Jun.
Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease (KFD) is characterized by its rare occurrence. Mostly prevalent among Asian women, KFD manifests with lymphadenopathy-affecting mostly cervical and rarely generalized or retroperitoneal regions-in addition to fever. It is a self-limited disease that resolves within 1-4 months, responding remarkably to glucocorticosteroids or hydroxychloroquine. However, some rare cases prove to be unresponsive to the previously mentioned therapies. Here is a description of a case of KFD affecting a 67-year-old Syrian woman with a history of hypothyroidism due to iodine-deficiency. The patient's initial clinical picture was malaise, fever, pericarditis and generalized lymphadenopathy. As treatment, she was given glucocorticosteroids with no significant response, while hydroxychloroquine proved to be partially effective. Until the date of this report, she has been receiving hydroxychloroquine with only slight clinical improvement. This case is proving to be resistant unlike most KFD cases that generally respond very well to treatment.
组织细胞坏死性淋巴结炎或菊池-藤本病(KFD)的特点是发病率罕见。KFD在亚洲女性中最为常见,除发热外,还表现为淋巴结病,主要累及颈部,很少累及全身或腹膜后区域。它是一种自限性疾病,在1-4个月内可自愈,对糖皮质激素或羟氯喹有显著反应。然而,一些罕见病例对上述治疗无反应。以下是一例KFD病例的描述,患者为一名67岁的叙利亚女性,有碘缺乏所致甲状腺功能减退病史。患者最初的临床表现为不适、发热、心包炎和全身淋巴结病。作为治疗,给予她糖皮质激素但无明显反应,而羟氯喹被证明部分有效。截至本报告发布之日,她一直在接受羟氯喹治疗,临床症状仅有轻微改善。与大多数对治疗反应良好的KFD病例不同,该病例表现出耐药性。