儿童色素减退型蕈样肉芽肿1例

A case of pediatric hypopigmented mycosis fungoides

报告色素减退型蕈样肉芽肿1例。患儿男,4岁。全身散发绿豆大小色素减退斑半年余,加重1个月。皮肤科检查:颈部、胸、背及四肢可见多发类圆形或圆形色素减退斑,境界清楚,未见融合,部分白斑中央见暗红色丘疹及斑片,表面干燥,覆盖白色鳞屑。左手背有两颗高于皮面黑色斑丘疹。皮损组织病理:表皮灶性角化不全,基底细胞液化变性,淋巴样细胞进入表皮内,少许核扭曲,见空晕,真皮浅层较多淋巴组织细胞苔藓样浸润,少许细胞轻异性。免疫组化提示CD20散在少量细胞(+)、CD79a散在少量细胞(+)、CD3(+)、CD4少量细胞(+)、CD5(+)、CD7大部分细胞(+)、CD8(+)、CD4与CD8比例为1∶4;CD30约30%细胞(+)、CD68散在少量细胞(+)、TIA-1(+)、Ki-67约30%细胞(+);CD7表达减弱。EBER原位杂交:(-)。根据临床表现、组织病理和免疫荧光,诊断为色素减退型蕈样肉芽肿。予盐酸氮芥溶液治疗,后失访。

A case of hypopigmented mycosis fungoides is reported.A 4-year-old boy had generalized mung bean sized hypopigmented spots for over 6 months,and aggravated 1 month.Dermatological examination showed multiple round hypopigmented spots on the neck,chest,back and limbs,with clear boundary and no mergence.Some white spots had dark red papules and patches in the center,with a dry surface and white scales.The left dorsal hand had two black macules.Histopathology of skin lesions showed focal parakeratosis,liquefaction of basal cells and lymphoid infiltration with twisted and clear halo nuclei in the epidermis.Lichenoid infiltration of lymphocytes with atypia was observed in the superficial dermis.Immunohistochemistry showed scattered positive of CD20,CD79a and CD4.CD3(+),CD5(+).Most cells were positive for CD7.CD8(+).The ratio of CD4 to CD8 was 1∶4.TIA-1(+).About 30% of cells were CD30(+),30% Ki-67(+),while a small number of cells were CD68(+).CD7 expression was decreased.EBER in situ hybridization was negative.According to the clinical manifestations,histopathology and immunofluorescence,the diagnosis of hypopigmented mycosis fungoides was made,and nitrogen mustard hydrochloride solution was given for the treatment.The case failed to be followed up.