种痘水疱病样皮肤T细胞淋巴瘤

Hydroa vacciniforme-like cutaneous T cell lymphoma

报告1例长期复发的种痘水疱病样皮肤T细胞淋巴瘤。患者女,47岁。从2岁开始反复发作水肿性红斑、水疱、坏死及痘疮样萎缩性瘢痕形成,冬轻夏重,主要累及面部和四肢。组织病理示真皮层内可见较多淋巴样细胞灶状和散在浸润,可见核分裂象。免疫组化检测显示淋巴样细胞CD2、CD3、CD4、CD5阳性,CD7、CD8少数细胞阳性,CD20、C1D56阴性,Ki-67约80%细胞阳性。原位杂交:很多细胞EBER阳性。诊断:种痘水疱病样皮肤T细胞淋巴瘤。

A case of long term relapsing hydroa vacciniforme-like cutaneous T cell lymphoma is reported.The patient was a 47-year-old woman. She came down with the disease since she was 2 years old,and the disease was mild in the winter and worse in the summer. The skin lesions were polymorphous, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and the lesions mainly involved the face and extremities. The viral serological examination showed Epstein- Barr virus （EBV） IgG and IgM were negative. Histopathology of the skin lesion showed diffuse and dense infiltration of lymphoid cells with anaplastic nuclei in the dermis as well as the subcutis. Immunohistochemistory showed CD2 （＋＋＋＋）,CD3 （＋＋＋＋）,CD4（＋＋）,CD5（＋＋＋＋）,CD7（＋）,CD8（＋）,Ki-67（＋＋＋＋）, CD20（-）, CD56（-）. EBER was positive in situ hybridization. A di- agnosis of hydroa vaceiniforme-like cutaneous T cell lymphoma was made.