Wen Peng-Fei, Zhou Qin-Tian, Wan Chuan, Liu Xiao-Yu, Xie Yao, Li Fan, Wang Lin
Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu 610041, China.
Department of Dermatology, the First Affiliated Hospital of PLA General Hospital, Beijing 100048, China.
Sichuan Da Xue Xue Bao Yi Xue Ban. 2019 Dec;50(6):930-934.
To explore the similarities and differences in clinical pathological features and gene rearrangement of lupus erythematosus profundus(LEP) and subcutaneous panniculitis-like T-cell lymphoma(SPTL).
We compared the clinical presentations, histopathology, immunophenotypical features and T-cell receptor (TCR) gene rearrangement findings of 9 cases of LEP and SPTL.
For clinical features, most patients of LEP occurred on head and face without systemic symptoms. LEP patients responded well to hydroxycholorquine treatment with good prognosis. Most patients of SPTL tended to lower extremities involvement and accompanied with systemic symptoms, the patients with disseminated lesions or hemophagocytic syndrome(HPS) showed poorer prognosis. For histopathology, LEP patients showed dense inflammatory infiltrate in the dermis consisting predominantly of lymphocytes with less numbers of plasma cells. However, the dermis was spared in SPTL, and rimming of adipocytes and erythrophagocytosis was observed in SPTL. Lymphocytes of LEP expressing CD4/CD8, as well as clusters of CD20. CD138-positive cells and scatter of CD123-positive cells were also observed in LEP. Tumor cells of SPTL were CD4/CD8, βF1, CD138 and CD123. The expression of TIA-1 or GrB was more favor in SPTL. Monoclonal T-cell receptor-γ gene rearrangement was found in 89% of SPTL patients while negative for LEP.
Base on different clinical and pathological features, it is easy to distinguish LEP from SPTL. However, a minority of lesions in LEP localize at subcutaneous tissue, which may turn to immunophenotypical and TCR gene rearrangement test for diagnosis.
探讨深部红斑狼疮（LEP）与皮下脂膜炎样T细胞淋巴瘤（SPTL）临床病理特征及基因重排的异同。
比较9例LEP和SPTL患者的临床表现、组织病理学、免疫表型特征及T细胞受体（TCR）基因重排结果。
临床特征方面，LEP患者多发生于头面部，无全身症状，对羟氯喹治疗反应良好，预后较好。SPTL患者多累及下肢，伴有全身症状，有播散性病变或噬血细胞综合征（HPS）的患者预后较差。组织病理学上，LEP患者真皮层有密集的炎症浸润，主要由淋巴细胞组成，浆细胞数量较少。而SPTL患者真皮层未受累，可见脂肪细胞边缘现象及噬红细胞现象。LEP患者的淋巴细胞表达CD4/CD8，同时也观察到CD20、CD138阳性细胞簇及散在的CD123阳性细胞。SPTL的肿瘤细胞表达CD4/CD8、βF1、CD138和CD123。TIA-1或颗粒酶B（GrB）的表达在SPTL中更常见。89%的SPTL患者检测到单克隆T细胞受体γ基因重排，而LEP患者为阴性。
基于不同的临床和病理特征，LEP与SPTL易于鉴别。然而，少数LEP病变位于皮下组织，可能需要进行免疫表型及TCR基因重排检测以明确诊断。
