幼儿肉芽肿性皮肤松弛症1例  

A Case of Granulomatous Slack Skin in A Child

作　　者：郑庆虎[1] 陈祯祥 伦宪鹏 潘芳[1] 王和平[1] 王浩[1] 

机构地区：[1]中国人民解放军第一五三中心医院皮肤科,河南郑州450042

出　　处：《中国皮肤性病学杂志》2017年第12期1342-1345,共4页The Chinese Journal of Dermatovenereology

摘　　要：患者男,5岁。下腹部、腹股沟、颈部皮肤粗糙伴松弛2年余。皮肤科情况:患者下腹部、会阴部色素沉着明显,可见大小不等的丘疹、浸润性斑块,皮肤萎缩、松弛、干燥明显,可见白色鳞屑。皮损组织病理示:淋巴细胞体积小,核无明显异型;多核巨细胞体积大,核多呈马蹄形或花环形排列。真皮层以胶原纤维为主,真皮浅层弹力纤维消失,真皮深层可见弹力纤维纤细、稀少,粗细不等,多数断裂。免疫组织化学示:LCA,CD3,CD4,CD43,CD45RO均为阳性。淋巴细胞克隆性Ig H基因重排检测结果示:FR(-),TCRΥ基因重排:JV(+),TCRΥ基因呈单克隆性基因重排,提示T淋巴细胞源性肿瘤。诊断:肉芽肿性皮肤松弛症。A 5-year-old boy presented with rough and slack skin on his neck,lower abdomen and groin for 2 years. Dermatological examination showed pigmentation and papules on his lower abdomen and perineum,the infiltrated plaques with white scalse could be seen. Histopathological examination showed sparse lymphocytes in the epithelium,a granuloma in the dermis,no pleomorphism of lymphocytes,multinuclear giant cells with nuclei forming hippocrepiform. And there were mainly collagen fibers in the dermis,while the elastic fibers disappeared in the superficial dermis,and were sparse,small or broken in the deeper dermis. Immunohistochemistry showed positive stain for LCA,CD3,CD4,CD43 and CD45RO. TCR recombinant showed IgH gene recombinant： FR（-）; TCRΥgene recombinant： JV（＋）,TCRΥ gene monoclonal recombinant,implied T cell lymphoma.A diagnosis of granulomatous slack skin（GSS）was made.

关 键 词：肉芽肿性皮肤松弛症 幼儿 皮肤T细胞淋巴瘤 

分 类 号：R739.5[医药卫生—肿瘤]