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一例皮下脂膜炎样 T 细胞淋巴瘤惰性病程,表现为 EBV 阳性并模拟皮损人工痕迹。

A case with an indolent course of subcutaneous panniculitis-like T-cell lymphoma demonstrating Epstein-Barr virus positivity and simulating dermatitis artefacta.

机构信息

2nd Dermatology Clinic, Ankara Numune Education and Research Hospital, Department of Pathology, Faculty of Medicine, Ankara University, Ankara, Turkey.

出版信息

Am J Clin Dermatol. 2010;11(2):147-50. doi: 10.2165/11311060-000000000-00000.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cytotoxic T-cell lymphoma. The disease has a poor prognosis in patients with the complication of hemophagocytic syndrome, especially in those with Epstein-Barr virus (EBV) positivity. A 20-year-old woman presented with multiple, itchy, purplish nodules and excoriations on both of her upper limbs. The histopathologic, immunohistochemical staining, and clonal T-cell receptor gene rearrangement examinations of skin lesions revealed the diagnosis of SPTCL. In situ hybridization performed for EBV was positive. There were no findings suggesting systemic involvement of SPTCL, or hemophagocytic syndrome. The lesions improved with systemic corticosteroid therapy and radiotherapy, with no recurrence. We present a patient with a protracted course of SPTCL in whom EBV positivity was demonstrated. This apparent conflict may be explained by geographic and ethnic variations in EBV infection. Further studies may shed light on the real relationship between EBV-RNA and the course of SPTCL.

摘要

皮下脂膜炎样 T 细胞淋巴瘤(SPTCL)是一种罕见的细胞毒性 T 细胞淋巴瘤。对于并发噬血细胞综合征的患者,尤其是 EBV 阳性的患者,疾病预后较差。一名 20 岁女性因上肢多发、瘙痒、紫色结节和抓痕就诊。皮肤病变的组织病理学、免疫组织化学染色和克隆 T 细胞受体基因重排检查均提示 SPTCL 的诊断。原位杂交 EBV 阳性。无 SPTCL 或噬血细胞综合征的系统受累的发现。病变经全身皮质类固醇治疗和放射治疗后改善,无复发。我们报告了一例 EBV 阳性的 SPTCL 患者,其病程迁延。这种明显的冲突可能是由于 EBV 感染的地域和种族差异所致。进一步的研究可能有助于阐明 EBV-RNA 与 SPTCL 病程之间的真正关系。

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