种痘水疱淋巴组织增殖性疾病1例报告

Hydroa vacciniforme lymphoproliferative disorder:a case report

本研究报道1例种痘水疱淋巴组织增殖性疾病(hydroa vacciniforme lymphoproliferative disorder,HVLPD)。患者为男性,首次就诊时21岁,颈面部、躯干反复出现丘疹、水疱样皮疹、出血结痂,血清EBV-DNA明显增高(2.88×107拷贝/mL)。患者2年内经2次皮肤活检。首次皮肤活检病理显示表皮棘层部分变性松解、表皮内水疱形成,部分表皮脱落,水泡及棘层内见多灶小脓肿,真皮内小淋巴细胞、浆细胞、组织细胞、嗜酸细胞片状浸润,淋巴细胞无明显不典型性,EBER原位杂交阴性,病理难以明确诊断。第2次皮肤活检病理显示,在光学显微镜下观察,活检标本表皮内可见水疱,真皮内有不典型淋巴样细胞围绕毛囊、汗腺及血管浸润;免疫组织化学检测示,淋巴样细胞CD3、CD5、CD4、CD8、granzyme B、TIA-1阳性,CD56、Perforin阴性,Ki-67增殖指数约为10%;EBER原位杂交阳性。患者接受对症治疗后1年余,皮疹加重,时有发热,伴左眼睑水肿,在外院行第3次皮肤活检,可见不典型淋巴样细胞浸润至皮下脂肪组织,Ki-67增殖指数为60%,提示疾病进展为EBER阳性T细胞淋巴瘤。我院予患者以吉西他滨+顺铂+地塞米松+培门冬酶化疗2个疗程后,其水肿消退、皮疹愈合。

In this study,we report a case of hydroa vacciniforme lymphoproliferative disorder(HVLPD).The patient,who was 21 years old at the time of initial consultation,suffering from recurrent papules,vesicular rashes,bleeding and black scabs on the neck,face and trunk.Serum EBV-DNA was significantly increased(2.88×107 copies/mL).The patient underwent skin biopsies twice within 2 years.The pathology of the first skin biopsy showed partial degeneration and loosening of the epidermal stratum spinosum,intraepidermal blister formation,partial epidermal detachment,and multifocal small abscesses seen in the blisters and stratum spinosum.Patchy infiltration of small lymphocytes,plasma cells,histiocytes,and eosinophils in the dermis,with no significant atypia of lymphocytes,EBER in situ hybridization was negative,which made it difficult to make a definitive diagnosis on pathology.The pathology of the second skin biopsy showed blisters visible within the patient′s epidermis,and atypical lymphoid cells infiltrate around the hair follicles,sweat glands and blood vessels in the dermis.The immunohistochemical analysis indicated that lymphoid cells were positive for CD3,CD5,CD4,CD8,granzyme B and TIA-1,while CD56 and Perforin were negative,and the proliferation rate of Ki-67 was approximately 10%.EBER was positive by in situ hybridization consistent with clinicopathologic features of HVLPD.More than 1 year after receiving symptomatic treatment,the patient′s rash worsened,with sometimes fever and left eyelid edema.The third skin biopsy performed in the other hospital showed that atypical lymphoid cells infiltrated the subcutaneous adipose tissue,and the proliferation rate of Ki-67 was 60%.The disease progressed to EBER-positive T-cell lymphoma.After 2 courses of chemotherapy with gemcitabine,cisplatin,dexamethasone and pegaspargase,the patient′s edema subsided and the rash healed.This report demonstrates the clinical and pathologic features of the disease during its evolution and progression,with a view to enriching its diagnostic and therapeutic experience.