【摘要】 目的介绍、普及种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病的相关内容。方法回顾我科2014年诊断的3例儿童期发病的种痘样水疱病样EB病毒感染相关淋巴细胞增生性疾病,分析其临床与病理特点。结果 3例患儿临床特点为面部、上肢曝光部位反复发生水疱、丘疹、溃疡、坏死、结痂,后皮疹累及躯干、下肢等非曝光部位,留有萎缩性瘢痕。疾病呈慢性进展。3例均伴发热、肝脾淋巴结受累。辅助检查:3例血液EB病毒Ig G抗体(Epstein-Barr virus-Ig G antibody,EBV-Ig G)均阳性、EB病毒脱氧核糖核酸(Deoxyribonucleic acid,DNA)复制活跃。组织病理:真皮及脂肪层弥漫或灶状分布较密集的淋巴细胞浸润,细胞有轻度异型性。免疫组化:CD4、CD8、CD56阳性表达不一;Ki-67阳性比例低,TIA、Gram B散在阳性。EB病毒编码RNA原位杂交(EBV-encoded RNA,EBER)均阳性。TCR基因重排阴性。结论本病组织病理特异性不高、免疫组化标志尚无标准、TCR基因重排阳性率低。因此,特征性的临床表现和EB病毒病原学检验的诊断权重要高于组织病理、免疫组化、TCR基因重排。是否进展为种痘样水疱病样皮肤T细胞淋巴瘤需密切随访。更多还原

【Abstract】 Objective To introduce a rare disease: Hydroa vacciniforme-like Epstein-Barr virus-associated lymphoproliferative disorder. Methods The clinical manifestation, laboratory examinations and tissue pathology of three cases of Hydroa vacciniforme-like Epstein-Barr virus-associated lymphoproliferative disorder diagnosed in 2014 were investigated and analyzed. Results All of three patients primarily presented with multiforme exanthema containing of blisters,papules, edema, blisters, ulcers, crusts, and scars on face and arms exposed to sun-light. Consequently, trunk and lower extremity suffered from similar skin rash described above. Fever was a mainly concomitant symptom. Also, mild lymphadenectasis, hepatomegaly or splenomegaly were found in physical examination. EBV-Ig G antibody in Peripheral blood was examined, and high replication of EBV genes was detected. The pathology of skin lesion exhibited that dermis and subcutaneous tissue were infiltrated by pervasive or dense lymphocytes with mild atypia. Immunohistochemical staining showed that part of the infiltrating lymphoid cells were respectively positive for CD4, or CD8, or CD56. Scattered cells were stained positive for Ki-67, TIA, Gram B. EBV-encoded RNA was examinated within tissue from skin biopsies of three patients by in-situ hybridization. Gene rearrangement of TCR was not detected. Conclusion Given the non-specific pathology changes, indefinitive markers of immunohistochemical staining and negative gene rearrangement of TCR, characteristic clinical manifestation and tests of EBV are prior to the above three examinations to establish this disease. Follow-ups are deliberately needed to detect whether hydroa vacciniforme-like cutaneous T cell lymphoma should be considered.更多还原