Montalvo Nelson, Redrobán Ligia
Servicio de Patología, Hospital Metropolitano, Quito, Ecuador.
Am J Dermatopathol. 2016 May;38(5):e57-9. doi: 10.1097/DAD.0000000000000485.
Hydroa vacciniforme-like cutaneous lymphoma is a very rare Epstein-Barr virus positive peripheral T-cell lymphoma affecting Asian and Hispanic children and young adults with a defective cytotoxic immune response to EBV predisposing to the development of the disease. We report on 2 Ecuadorian patients with papulovesicular and ulcerated crusted lesions on the face, upper and lower extremities and abdomen, with aggressive clinical course and, in one case, a fatal outcome. The histological and molecular profiles (immunohistochemistry and in situ hybridization) established a diagnosis of hydroa vacciniforme-like Epstein-Barr virus-encoded small RNAs + cutaneous T-cell lymphoma in both cases.
种痘样水疱病样皮肤淋巴瘤是一种非常罕见的爱泼斯坦-巴尔病毒阳性外周T细胞淋巴瘤，影响亚洲和西班牙裔儿童及年轻成人，其对EBV的细胞毒性免疫反应存在缺陷，易引发该疾病。我们报告了2例厄瓜多尔患者，他们的面部、上肢、下肢和腹部出现丘疹水疱性及溃疡结痂性皮损，临床病程进展迅速，其中1例患者死亡。通过组织学和分子特征（免疫组织化学和原位杂交），这2例患者均被诊断为种痘样水疱病样爱泼斯坦-巴尔病毒编码小RNA阳性皮肤T细胞淋巴瘤。
