中华皮肤科杂志

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牛痘样水疱病样原发性皮肤CD8阳性T细胞淋巴瘤一例

冯素英, 靳培英, 曾学思, 姜祎群   

  1. 中国医学科学院皮肤病研究所, 南京, 210042
  • 收稿日期:2006-11-24 出版日期:2007-10-15 发布日期:2007-10-15
  • 通讯作者: 靳培英,email:jinpeiying@medmail.com.cn E-mail:jinpeiying@medmail.com.cn

A case of hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma

FENG Su-ying, JIN Pei-ying, ZENG Xue-si, JIANG Yi-qun   

  1. Institute of Dermatology, Chinese Academy of Medical Sciences, Nanjing 210042, China
  • Received:2006-11-24 Online:2007-10-15 Published:2007-10-15

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摘要： 一例13岁女孩面部和四肢为主反复出现红斑、丘疹、水疱样皮损9年,皮疹形态与牛痘水疱病相似(hydroa vacciniforme,HV)。在病情急性进展期,伴高热,体温39℃以上。皮肤科检查:面部水肿,面部和四肢较多的萎缩性圆形和椭网形痘疮样瘢痕,散在红斑、丘疹、坏死和结痂;四肢数个水肿性红色斑块,中心有圆形破溃面。组织学上,在真皮的血管和附属器周围,可见大量表达CD8异形淋巴细胞浸润,诊断CD8+皮肤T细胞淋巴瘤。糖皮质激素和免疫抑制剂可使病情缓解,随诊至今,无系统受累情况。

关键词: 淋巴瘤, T细胞, 皮肤, CD8阳性T淋巴细胞, 牛痘样水疱症

Abstract: A 13-year-old girl presented with a 9-year history of hydroa vacciniforme(HV)-like eruption,including erythema,papules and vesicles,mainly distributed at the face and extremities.A high fever(>39℃)developed at the progressive stage of this disease.Physical examination showed variously sized,round or oval,atrophic and variola-like scars along with scattered erythematous patches,papules, necrosis and crusts on the face and extremities.The face was edematous,and there were some edematous and erythematous plaques with a necrotic center on the legs and arms.Histological examination revealed a massive infiltration with atypical CD8+ lymphocytes around the vessels and appendages in dermis.A diagnosis of CD8+ cutaneous T-cell lymphoma(CTCL)was made.Glucocorticoid and immunosuppressants were effective in controlling the condition.Up to the time of the writing,there has not been any definite evidence of systemic involvement.

Key words: Lymphoma, T-cell, cutaneous, CD8-positive T-lymphocytes, Hydroa vacciniforme