摘要
目的:探讨种痘样水疱病样T淋巴细胞增生病的诊断和治疗方法,提高对该病的认识。方法:回顾分析1例种痘样水疱病样T淋巴细胞增生性疾病患者的临床表现、辅助检查、诊疗过程及预后等临床资料,并对相关文献进行复习。结果:18岁男性患者,2年来反复出现红斑、水疱、结痂和萎缩性瘢痕等症状,皮损分布于日光暴露部位,并伴有发热、肝脾大等全身症状。皮损组织学检查显示:真皮附件周围有大量淋巴细胞浸润,淋巴细胞成团聚集,偶可见淋巴细胞的细胞核分裂变形。免疫组化检查:皮损部位CD3、CD45RO和EB病毒IgM抗体反应为阳性,而CD20、CD79a、CD56、CD30、Ki-67、TIA-1抗体反应为阴性。该患者通过糖皮质激素和免疫调节药物治疗后,病情明显缓解。结论:种痘样水疱病样T淋巴细胞增生性疾病临床罕见,与EB病毒感染密切相关,如患者日光暴露部位的皮肤反复出现丘疹、水疱、坏死,并伴有发热,应高度怀疑该病可能性;糖皮质激素联合免疫调节药物可控制该病的症状,但应进行长期随访。
Objective： A cases of hydroa vacciniforme-like Epstein-Barr virus-associated lymphoproliferative disorder is re- ported. Methods： One case of vacciniforme-like T lymphocyte proliferative disease was studied. The clinical manifestations, laboratory examinations, diagnosis and treatment of the disease were retrospectively analyzed, and the relevant literature was reviewed. Results： The patient was an 18- year-old man, he presented with erythema, blisters, crusts and atrophic scar with fever for 2 years. The disease relapsed for several times, and the skin lesions distributed on the sun exposed areas, accompa- nied by fever and systemic involvement, such as hepatosplenomegaly. Histopathology of the skin lesion revealed large amounts of infiltration of lymphocytes around the dermal appendages, gathered in clusters with occasional karyomitosis and distorted nu- clei. Immunohistochemical study showed positive stain for CD3, CD45RO and Epstein-Barr virus IgM antibody, while negative for CD20, CD79a, CD56, CD30, Ki-67 and TIA-1. The patient improved after treatment with glucocorticoid and immunomod- ulatory drugs. Conclusions： Vacciniform-like vesicular disease of T lymphocyte proliferative disease is uncommon, the disease is closely related to EB virus. When a patient presented with recurrent papules, blisters, and necrosis on the sun exposed parts of the body and accompanied by fever, the diagnosis of hydroa vacciniforme-like T lymphoproliferative disease should be highly suspected. Although glucocorticoids combined with immunomodulatory drugs can control the symptoms, long-term follow- up is strongly recommended.
出处
《临床皮肤科杂志》
CAS
CSCD
北大核心
2013年第3期172-174,共3页
Journal of Clinical Dermatology
关键词
T淋巴细胞增生
种痘样水疱病
EB病毒
T lymphoproliferative, hydroa vacciniforme
Epstein-Barr virus
