Ghosn Samer, Bahhady Ruba, Mahfouz Rami, Abbas Ossama, Kibbi Abdul Ghani, Saad Rana, Taher Ali
Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon.
Am J Dermatopathol. 2009 Dec;31(8):814-8. doi: 10.1097/DAD.0b013e3181acedf8.
Kimura disease (KD) is a rare condition that predominantly affects young middle-aged Asian men. It is classically characterized by tumors in the head and neck region with associated eosinophilia and elevated serum immunoglobulin E levels. The exact pathogenesis of this condition remains unknown. Although some regard it as a reactive condition, others believe that it is a T-cell-mediated disease. T-cell clonality has been recently demonstrated in a few cases. We report a 37-year-old Lebanese woman who had both KD and mycosis fungoides (MF). T-cell receptor gene rearrangement studies using 2 different techniques did not detect any clonality for T-cell receptor in both KD and MF lesions. Due to the presence on histology of lymphoid follicles and the persistent high serum immunoglobulin E levels, we elected to attempt treatment with rituximab. Although the KD lesions persisted, they became softer and less nodular. As for the MF lesions, they flared transiently and then exhibited a sustained improvement over a follow-up period of 1 year.
木村病（KD）是一种罕见疾病，主要影响年轻的中年亚洲男性。其典型特征是头颈部出现肿瘤，并伴有嗜酸性粒细胞增多和血清免疫球蛋白E水平升高。这种疾病的确切发病机制尚不清楚。尽管有些人认为它是一种反应性疾病，但另一些人则认为它是一种T细胞介导的疾病。最近在少数病例中证实了T细胞克隆性。我们报告了一名37岁的黎巴嫩女性，她同时患有木村病和蕈样肉芽肿（MF）。使用两种不同技术进行的T细胞受体基因重排研究未在木村病和蕈样肉芽肿病变中检测到T细胞受体的任何克隆性。由于组织学上存在淋巴滤泡且血清免疫球蛋白E水平持续升高，我们选择尝试使用利妥昔单抗进行治疗。尽管木村病病变持续存在，但它们变得更软且结节性减少。至于蕈样肉芽肿病变，它们短暂发作，然后在1年的随访期内持续改善。
