摘要
目的分析9例色素减退性蕈样肉芽肿(HMF)的临床及病理学特点。方法回顾性分析2014—2018年在杭州市第三人民医院皮肤科经临床及组织病理学确诊的9例HMF患者的临床、病理表现及随访情况。结果9例患者中男7例,女2例;年龄6~29岁,平均12.4岁;病程最短6个月,最长3年,平均20.7个月。所有皮损均表现为色素减退斑片,边界不清,形状欠规则,部分色素减退斑融合,2例色素减退斑上覆鳞屑。Wood灯下色素减退斑呈灰白色,荧光弱;皮肤镜下皮损处可见白色鳞屑,精子样、点状或短线状血管模式;反射式共聚焦显微镜下可见基底层色素轻度减退,未见明显色素缺失,表皮及真表皮交界处可见高折光异形细胞。9例组织病理均表现为异形淋巴细胞亲表皮现象,部分可见细胞聚集成Pautrier微脓肿;免疫组化染色显示细胞表型均符合T细胞表型,以CD8+T细胞浸润为主。9例患者采用窄谱中波紫外线(NB-UVB)治疗,均达临床痊愈。平均随访23.8个月,2例复发,再次行NB-UVB治疗,均达临床痊愈。结论HMF病情进展缓慢,NB-UVB治疗效果较好,患者达临床痊愈后存在复发可能,均需长期随访。
Objective To analyze clinical and pathological characteristics of 9 cases of hypopigmented mycosis fungoides(HMF).Methods Nine patients with clinically and histopathologically confirmed HMF were collected at Department of Dermatology,Hangzhou Third People's Hospital from 2014 to 2018,and their clinical and pathological manifestations and follow-up findings were retrospectively analyzed.Results Among the 9 patients,there were 7 males and 2 females;the age ranged from 6 to 29 years(average,12.4 years);the duration of disease varied from 6 months to 3 years(average,20.7 months).All skin lesions manifested as poorly demarcated,irregular,hypopigmented patches,some hypopigmented patches were confluent,and hypopigmented patches were covered with scales in 2 cases.Wood′s lamp examination of hypopigmented patches showed a grayish-white color with weak fluorescence.Dermoscopic images showed white scales on the lesions and blood vessels distributed in spermatozoa-like,punctiform or short linear patterns.Reflectance confocal microscopy showed mild hypopigmentation in the basal layer without obvious depigmentation,and highly-refractive atypical cells at the dermo-epidermal junction.Histopathological examination of the 9 cases showed epidermotropic phenomenon of atypical lymphocytes,and some cells clustered and formed Pautrier′s microabscess,immunohistochemical staining revealed that all the cells expressed T cell phenotypes,and were mainly infiltrating CD8+T cells.The 9 patients were treated with narrow-band ultraviolet B(NB-UVB),and all of them achieved clinical complete response.During a follow-up of 23.8 months on average,2 patients experienced recurrence,and they achieved clinical complete response again after NB-UVB treatment.Conclusions HMF progresses slowly,and NB-UVB treatment is effective.There is the possibility of recurrence after clinical recovery in patients with HMF,and a long-term follow-up is needed.
作者
乔刚
雷杰豪
许爱娥
Qiao Gang;Lei Jiehao;Xu Ai′e(Department of Dermatology,Hangzhou Third People′s Hospital,Hangzhou 310009,China)
出处
《中华皮肤科杂志》
CAS
CSCD
北大核心
2020年第4期292-295,共4页
Chinese Journal of Dermatology
基金
国家自然科学基金(81773335)。
关键词
蕈样真菌病
色素沉着不足
皮肤表现
病理过程
紫外线疗法
治疗结果
Mycosis fungoides
Hypopigmentation
Skin manifestations
Pathologic processes
Ultraviolet therapy
Treatment outcome
