色素性紫癜样蕈样肉芽肿1例

A case of pigmented purpuric mycosis fungoides

报告1例色素性紫癜样蕈样肉芽肿。患者男,37岁,左大腿紫红色斑片10年,泛发加重1年。皮肤科检查:左大腿外侧大片紫红斑,边界欠清,压之不褪色,伴色素沉着及轻度苔藓样变;双侧耳后、躯干散在类似皮损。皮损组织病理检查:真皮层弥漫单一核细胞浸润,伴红细胞外渗,部分单一核细胞移入表皮,形成Pautrier微脓疡,细胞核周可见空晕。免疫组化:CD3及CD4均(+),CD8(±),增殖核抗原(Ki-67)(20%+),CD7、CD20、CD79a、CD56、CD30、颗粒酶(Granzyme)B、间变性淋巴瘤激酶(ALK)及EB病毒编码的小RNA(EBER)均(-)。基因重排:TCRG克隆性重排为阳性。诊断:色素性紫癜样蕈样肉芽肿。

A case of pigmented purpuric mycosis fungoides is reported.A 37-year-old male patient presented with a purple-red patch on his left thigh for 10 years,and generalized exacerbation for 1 year.Physical examination showed a large area of purple-red patch on the outside of the left thigh,with an unclear border.The patch did not fade after pressing,with pigmentation,desquamation,and mild mossy changes.Similar rashes could be seen behind both ears and on the trunk.Derma tological examination showed diffuse mononuclear cell infiltration with red blood cell extravasation in the dermis,and some mononuclear cells migrated into the epidermis,forming a Pautrier microabscess,with a hollow halo around the nucleus.Immunohistochemical investigation demonstrated that the tumor cells were positive for CD3,CD4,CD8(Scattered),Ki-67(about 20%),and negative for CD7,CD20,CD79 a,CD56,CD30,Granzyme B,anaplastic lymphoma kinase(ALK),EBV-encoded RNA(EBER).Gene rearrangement showed that TCRG clonal rearrangement was positive.The diagnosis of pigmented purpuric mycosis fungoides was made.