皮肤色素减退性淋巴增殖性疾病41例临床病理分析

Cutaneous hypopigmented lymphoproliferative disorders:a clinicopathological study of 41 cases

目的探讨色素减退性蕈样肉芽肿(HMF)与色素减退性界面T细胞异常增生(HITCD)的临床与病理特征。方法收集2015年1月至2020年9月在杭州市第三人民医院皮肤科就诊、具有完整临床病理资料的皮肤色素减退性淋巴增殖性疾病患者41例,分析临床病理及免疫表型特征。符合正态分布的定量数据使用t检验,分类数据采用卡方检验或Fisher精确检验,两组间等级数据比较采用秩和检验。结果41例患者临床均表现为不规则色素减退斑,可伴有红斑或糠状鳞屑。病理特征:21例表现为不典型淋巴样细胞向表皮浸润和聚集,符合典型蕈样肉芽肿病理学特征,诊断为HMF;20例表现为基底层空泡变性伴有淋巴样细胞浸润以及轻度嗜表皮现象,诊断为HITCD。免疫细胞表型均符合T细胞表型,14例(67%)HMF与13例(65%)HTCD表皮淋巴细胞以CD8表型为主。表皮中,HMF组总淋巴细胞数量多于HITCD组(t=1.81,P=0.012);真皮中,HMF组CD4^(+)淋巴细胞(t=2.64,P=0.012)、CD8^(+)淋巴细胞(t=1.51,P=0.002)以及总淋巴细胞数量(t=2.60,P=0.001)均多于HITCD组。所有患者均予窄谱中波紫外线照射治疗,完成随访34例,30例(包括所有HITCD)皮损消退未复发,4例HMF皮损未完全消退。结论HITCD与HMF病理特征不同,且呈良性生物学行为,应作为一类独立疾病区别于HMF,单一光疗可获良效。

Objective To investigate clinicopathological features of hypopigmented mycosis fungoides(HMF)and hypopigmented interface T-cell dyscrasia(HITCD).Methods A total of 41 patients with cutaneous hypopigmented lymphoproliferative diseases,who had complete clinicopathological data,were collected from Department of Dermatology,the Third People's Hospital of Hangzhou from January 2015 to September 2020,and the clinicopathological and immunophenotypic features were analyzed.Comparisons of normally distributed measurement data were carried out using t test,comparisons of categorical data using Chi-square test or Fisher's exact test,and comparisons of ranked data between 2 groups using rank-sum test.Results All of the 41 patients clinically presented with irregular hypopigmentation,some of which was accompanied by erythema or furfuraceous scales.In terms of pathological features,21 patients showed infiltration and aggregation of atypical lymphoid cells in the epidermis,which was consistent with typical pathological features of mycosis fungoides,and they were diagnosed with HMF;20 patients showed vacuolar degeneration of the basal layer,accompanied by infiltration of lymphoid cells and mild epidermotropism,and they were diagnosed with HITCD.All immune cells expressed T-cell phenotype,and epidermal lymphocytes expressed a CD8-dominated phenotype in 14(67%)cases of HMF and 13(65%)of HITCD.In the epidermis,the total number of lymphocytes was significantly higher in the HMF group than in the HITCD group(t=1.81,P=0.012);in the dermis,the number of CD4^(+) lymphocytes and CD8^(+)lymphocytes,and the total number of lymphocytes were all significantly higher in the HMF group than in the HITCD group(t=2.64,1.51,2.60,P=0.012,0.002,0.001,respectively).All patients were treated with narrow-band ultraviolet B radiation.Among 34 patients who completed the follow-up,30 achieved complete clearance of skin lesions without recurrence,including all patients with HITCD,and 4 with HMF achieved partial,regression of the lesions.Conclusions Compared with HMF,HITCD presents different pathological characteristics and benign biological behaviors.Thus,HITCD should be distinguished from HMF as an independent disease.Phototherapy alone is effective for the treatment of HITCD.