Liu Jie, Wang Bao-Xi, Qu Tao, Liu Yue-Hua, Fang Kai, Yan Yan
Department of Dermatology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2007 Apr;29(2):174-80.
To update the clinical characteristics of mycosis fungoides in Chinese patients.
We retrospectively analyzed the clinical data of 51 patients (29 men and 22 women) with mycosis fungoides in PUMC hospital from 1984 to 2006, to determine the ages at diagnosis, clinicopathologic characteristics of skin lesions, systemic manifestation, misdiagnosis and treatment of these patients.
The mean age was (44.24 +/- 2.05) years at the diagnosis. Most patients were characterized by the typical evolution of patches, plaques and tumors, with some variations and subtypes. Clinical manifestations included generalized lesions (68.6%) and itchy (70.6%). Epidermotropism (68.6%) and Pautrier's microabscesses (52.9%) were common histopathologic features. The positive rate of T-cell receptor gene rearrangement was 81.3%, and was independent of the histological features. Previous misdiagnosis rate was 64.7%. Skin-targeted therapies and biologic therapies were effective approaches to relieve the skin rash at early stage, and combined chemotherapy was typically applied in more advanced cases.
Mycosis fungoides has various clinical characteristics and careful differential diagnosis should be made in clinical practice.
更新中国蕈样肉芽肿患者的临床特征。
我们回顾性分析了1984年至2006年在协和医院确诊的51例蕈样肉芽肿患者（29例男性，22例女性）的临床资料，以确定这些患者的诊断年龄、皮肤病变的临床病理特征、全身表现、误诊情况及治疗方法。
确诊时的平均年龄为（44.24±2.05）岁。大多数患者表现为典型的斑片、斑块和肿瘤进展过程，伴有一些变异和亚型。临床表现包括全身病变（68.6%）和瘙痒（70.6%）。亲表皮现象（68.6%）和Pautrier微脓肿（52.9%）是常见的组织病理学特征。T细胞受体基因重排的阳性率为81.3%，且与组织学特征无关。既往误诊率为64.7%。以皮肤为靶点的治疗和生物治疗是早期缓解皮疹的有效方法，联合化疗通常用于病情更晚期的病例。
蕈样肉芽肿具有多种临床特征，临床实践中应仔细进行鉴别诊断。
