中国麻风皮肤病杂志 ›› 2023, Vol. 39 ›› Issue (11): 813-817.doi: 10.12144/zgmfskin202311813

• 临床研究 • 上一篇    下一篇

14例儿童色素减退型蕈样肉芽肿诊疗分析

夏云,胡枫,姜倩,蒋芮理,陈柳青   

  1. 武汉市第一医院皮肤科，湖北武汉，430022
  • 出版日期:2023-11-15 发布日期:2023-10-27

Diagnosis and treatment of 14 children with hypopigmented mycosis fungoides

XIA Yun, HU Feng, JIANG Qian, JIANG Ruili, CHEN Liuqing   

  1. Department of Dermatology, Wuhan No.1 Hospital, Wuhan 430022, China
  • Online:2023-11-15 Published:2023-10-27

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摘要： 回顾性分析2015年7月至2020年7月在武汉市第一医院皮肤科经临床和病理确诊的14例儿童色素减退型蕈样肉芽肿（HMF）临床、影像、病理、治疗及随访资料。14例患儿中男5例，女9例。诊断年龄4~13岁，发病年龄3~12岁，病程9个月~7年。皮损表现为大小不等、不规则形或类圆形，边界欠清晰的色素减退斑，局部融合。皮肤镜下见色素减退呈波点状、斑马样和网格状模式，血管呈点状、精子样、短细线状和星状血管结构模式。反射式共聚焦显微镜（RCM）下色素减退斑均表现为基底层色素轻度减退，未见明显色素缺失，真表皮交界处及真皮浅层可见较多高折光异形细胞。皮损组织病理示棘层下部和基底层可见异形淋巴细胞浸润，核深染，部分扭曲呈脑回状，部分聚集呈Pautrier微脓疡。免疫组化：CD3均阳性，CD20均阴性，4例仅CD8阳性，10例CD8、CD4均阳性。12例患儿采用NB-UVB照射治疗，8例完全消退，4例部分消退，3例复发再次照射后完全消退。2例分别给予糠酸莫米松和他克莫司治疗，6个月和8个月后消退面积＞80%。本研究显示皮肤影像有一定辅助诊断价值，NB-UVB治疗HMF安全有效，HMF预后一般良好，但仍需警惕治愈后有疾病复发及进展可能，需进行长期随访。

关键词: 儿童, 色素减退型蕈样肉芽肿, 皮肤病理, 皮肤影像, 窄谱中波紫外线

Abstract: The clinical, imaging, pathological, therapeutic and follow-up data of 14 children (5 males and 9 females) of hypopigmented mycosis fungoides (HMF) in the department of dermatology of our hospital from July 2015 to July 2020 were retrospectively analyzed. The result showed the age at diagnosis was from 4 to 13 years old, the age of onset was from 3 to 12 years old, and the course of disease was 9 months -7 years. The lesions presented as hypopigmentation spots of different sizes, irregular shape or almost round shape with poorly defined boundaries and local fusion. Under dermoscopy, hypopigmentation showed spot-like, zebrar-like and grid patterns, and blood vessels showed spot-like, spermatoid, short fine line and stellate vascular structure patterns. Under reflection confocal microscopy (RCM), hypopigmentation spots all showed mild hypopigmentation in the basal layer. No obvious pigment loss was observed, and more highly refraction heteromorphic cells were observed at the junction of the true epidermis and in the superficial layer of the dermis. Histopathologic examination of the skin lesions revealed the infiltration of irregular lymphocytes in the subspinous layer and basal layer, deep staining of the nuclei, some of them twisted to gyrus shape, and some of them clustered to Pautrier microabscess. Immunohistochemistry showed positive for CD3, CD8, both CD8 and CD4 in 14, 4, 10 cases and negative for CD20 in 14 cases. Twelve cases were treated with NB-UVB irradiation, 8 cases completely resolved, 4 cases partially resolved, and 3 cases recurred and completely resolved after re-irradiation. This study shows skin imaging has a certain auxiliary diagnostic value. Two patients were treated with topical mometasone furoate cream and tacrolimus cream, respectively, and 80% of the skin lesions were subside after 6 and 8 months. The treatment of NB-UVB is safe and effective, and the prognosis of HMF is generally good. However, it is necessary to be alert to the possibility of relapse and progress of HMF after cure, and long-term follow-up is required.

Key words: children, hypopigmented mycosis fungoides, cutaneous histopathology, skin image, narrow band ultraviolet B