Gonzalez Estrella Garcia, Selvi Enrico, Lorenzini Sauro, Maggio Roberta, Mannucci Susanna, Galeazzi Mauro, Marcolongo Roberto
Section of Rheumatology, Clinical Medicine and Immunological Science, University of Siena, Siena, Italy.
Clin Rheumatol. 2007 Feb;26(2):244-6. doi: 10.1007/s10067-005-0125-x. Epub 2006 Jan 11.
We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL), associated with macrophage activation syndrome, mimicking a lupus erythematosus panniculitis (LEP). A 29-year-old woman presented with high fever, general malaise, nausea, vomiting, and subcutaneous nodules and ulcerating lesions located on the lower extremities. The histopathology showed an infiltration of the panniculus, mostly involving fat, and periadnexial and perivascular structures consistent with lymphocytic lobular panniculitis (LLP). LLP is a shared feature of LEP and SPTCL. The immunophenotyping of the cell infiltrate was crucial for a correct diagnosis.
我们报告一例皮下脂膜炎样T细胞淋巴瘤（SPTCL），其与巨噬细胞活化综合征相关，酷似红斑狼疮性脂膜炎（LEP）。一名29岁女性，出现高热、全身不适、恶心、呕吐，以及位于下肢的皮下结节和溃疡性病变。组织病理学显示脂膜浸润，主要累及脂肪，以及与淋巴细胞性小叶性脂膜炎（LLP）一致的附件周围和血管周围结构。LLP是LEP和SPTCL的共同特征。细胞浸润的免疫表型分析对正确诊断至关重要。
