Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
J Dermatol. 2021 Sep;48(9):1315-1326. doi: 10.1111/1346-8138.15944. Epub 2021 May 13.
Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare cutaneous disease associated with Epstein-Barr virus infection. We retrospectively analyzed the clinical presentation, histopathological characteristics, and prognostic study of HV-LPD in 24 Chinese patients. All patients presented with recurrent papulovesicular and necrotic eruptions on the face, neck, and extremities, with 11 showing systemic symptoms. Twenty patients were diagnosed with HV-LPD in childhood (age < 18 years) and four in adulthood (age ≥ 18 years). The median age at diagnosis was 8.5 years old (range, 2-50). Histopathology revealed variably dense lymphocyte infiltration throughout the dermis. All cases were strongly positive for CD3 and Epstein-Barr encoding region based on in situ hybridization. Of 18 cases with a T-cell phenotype, 15 harbored monoclonal rearrangements in T-cell receptor (TCR) genes. Four cases with a natural killer cell phenotype carried polyclonal rearrangements in TCR genes. Among 24 patients, eight (33.3%) received chemotherapy, two (8.3%) allogeneic hematopoietic stem cell transplantation, and both are currently alive without disease. The median follow-up period was 24 months (range, 7-120) and 23 patients were available: 15 (62.5%) were alive, and eight (33.3%) had died. Fourteen cases had a relapse of disease and three developed lymphoma within 24 months of diagnosis. The mean survival time of childhood-onset patients was longer than that of adult-onset patients (36.4 vs. 20.8 months). In summary, the wide clinical course and representative presentation of cases in our center reflect the pedigree characteristics of HV-LPD. Allogeneic hematopoietic stem cell transplantation should be a preferred choice for relapse and refractory patients due to the poor effect of chemotherapy. Adult-onset and high serum EBV DNA loads may indicate an increased risk of aggressive disease in patients with HV-LPD.
水疱疹样淋巴组织增生性疾病（HV-LPD）是一种罕见的与 EBV 感染相关的皮肤疾病。我们回顾性分析了 24 例中国 HV-LPD 患者的临床表现、组织病理学特征和预后研究。所有患者均表现为面部、颈部和四肢反复发作的水疱性和坏死性皮疹，11 例伴有全身症状。20 例患者在儿童期（年龄<18 岁）和 4 例在成年期（年龄≥18 岁）诊断为 HV-LPD。诊断时的中位年龄为 8.5 岁（范围：2-50 岁）。组织病理学显示真皮内弥漫性淋巴细胞浸润。所有病例均通过原位杂交显示 CD3 和 EBV 编码区强阳性。在 18 例 T 细胞表型病例中，15 例存在 TCR 基因的单克隆重排。4 例自然杀伤细胞表型病例存在 TCR 基因的多克隆重排。在 24 例患者中，8 例（33.3%）接受化疗，2 例（8.3%）接受异基因造血干细胞移植，目前均无病生存。中位随访时间为 24 个月（范围：7-120 个月），23 例患者可评估：15 例（62.5%）存活，8 例（33.3%）死亡。14 例疾病复发，3 例诊断后 24 个月内发生淋巴瘤。儿童发病患者的平均生存时间长于成人发病患者（36.4 个月 vs. 20.8 个月）。总之，本中心广泛的临床病程和代表性病例反映了 HV-LPD 的家族特征。由于化疗效果不佳，异基因造血干细胞移植应为复发和难治性患者的首选。成人发病和高血清 EBV DNA 载量可能表明 HV-LPD 患者疾病侵袭性增加的风险增加。
