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目的总结1例牛痘样水疱病样皮肤淋巴瘤的临床特点和组织病理学特征,旨在提高对这一疾病的认识。方法收集患者的临床资料,观察其组织学形态并行免疫组化和EB病毒原位杂交检测。结果患儿女性,7岁。2年内面部、四肢及躯干部反复出现红斑、水疱,疱破后结痂,遗留痘疮样瘢痕,伴长期间断发热。皮肤活检:表皮内见多房水疱,水疱内、真皮层及皮下脂肪组织可见中等大小异型淋巴细胞弥漫浸润,部分围绕皮肤附属器及血管呈小叶状分布,伴血管中心性浸润和坏死。肿瘤细胞间穿插分布少量嗜酸性粒细胞和组织细胞。免疫组化肿瘤细胞CD3、CD45RO、CD8、TIA-1、穿孔素、粒酶B和LMP-1(+),而CD56(-);原位杂交EBER(+)。结论牛痘样水疱病样皮肤淋巴瘤是好发于儿童的罕见皮肤淋巴瘤,诊断及鉴别诊断需结合临床皮疹特点、组织病理学形态、免疫组化及原位杂交综合分析。
Objective To summarize the clinical features and histopathological features of a case of vaccinia-like blister-like cutaneous lymphoma in order to raise awareness of the disease. Methods The clinical data of patients were collected and their histological morphology was observed by immunohistochemistry and Epstein-Barr virus in situ hybridization. Results Children with children, 7 years old. 2 years, facial, limbs and torso repeated erythema, blisters, blisters after crusting, leaving a pox-like scar, with long-term intermittent fever. Skin biopsy: see the epidermis, multi-room blisters, blisters, dermis and subcutaneous adipose tissue can be seen diffuse infiltration of medium-sized atypical lymphocytes, some appendages and vessels around the skin showed lobular distribution, with vascular central infiltration and necrosis. Interstitial tumor cells interspersed with a small amount of eosinophils and tissue cells. Immunohistochemical tumor cells CD3, CD45RO, CD8, TIA-1, perforin, granzyme B and LMP-1 (+), while CD56 (-); in situ hybridization EBER (+). Conclusions Vaccinia-like blister-like cutaneous lymphoma is a rare cutaneous lymphoma in children. The diagnosis and differential diagnosis should be combined with clinical rash, histopathology, immunohistochemistry and in situ hybridization.