Su Ting, Wang Wentong, Xiong Xixi, Zhang Meihua, Lu Yan, Su Zhonglan
Department of Dermatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, People's Republic of China.
Clin Cosmet Investig Dermatol. 2024 May 30;17:1267-1274. doi: 10.2147/CCID.S460784. eCollection 2024.
Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, mimicking hemophagocytic lymphohistiocytosis (HLH) and subcutaneous panniculitis-like T-cell lymphoma (SPTCL).
To establish the clinicopathological features and response to treatment of CHP and evaluate the prognosis of patients and guide therapy based on the current state of knowledge.
Clinical, laboratory, histopathological, and outcome data of 12 patients with CHP were retrospectively collected between 2009 and 2022.
All the patients presented with plaques or nodules, mostly located in the lower extremities (11/12). Fewer cases involved systemic symptoms (9/12) and laboratory abnormalities (6/12), and none were positive for serum Epstein-Barr virus (EBV)-DNA. Histopathological examination revealed mixed septal and lobular inflammatory infiltration of histiocytes and lymphocytes. Large or atypical lymphocytes were rarely present (2/12). In some patients, varying proportions of plasma cells, neutrophils, and eosinophils were observed. The extent of histocytophagy was mild (9/12), moderate (2/12), and severe (1/12). HLH was not observed in any of our cases, none of which were fatal.
The uniqueness of our study lies in the presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders (CTD) and streptococcal infections. Our study reveals that EBV-negative CHP tends to a better prognosis than previously research, filling the gap in the much-needed details of CHP in the Chinese population. Moreover, CHP may present as a reactive process in combined primary diseases; further studies are required to validate these findings.
噬血细胞性组织细胞性脂膜炎（CHP）是一种罕见的脂膜炎，伴有全身症状，其特征为皮下脂肪组织被外观良性的T淋巴细胞和吞噬性组织细胞浸润，类似噬血细胞性淋巴组织细胞增生症（HLH）和皮下脂膜炎样T细胞淋巴瘤（SPTCL）。
基于目前的知识状况，确立CHP的临床病理特征及对治疗的反应，评估患者预后并指导治疗。
回顾性收集了2009年至2022年间12例CHP患者的临床、实验室、组织病理学及结局数据。
所有患者均表现为斑块或结节，大多位于下肢（11/12）。较少病例出现全身症状（9/12）和实验室异常（6/12），血清EB病毒（EBV）-DNA均为阴性。组织病理学检查显示组织细胞和淋巴细胞呈混合性间隔和小叶性炎症浸润。很少出现大或非典型淋巴细胞（2/12）。在一些患者中，观察到不同比例的浆细胞、中性粒细胞和嗜酸性粒细胞。组织细胞吞噬程度为轻度（9/12）、中度（2/12）和重度（1/12）。我们的病例中未观察到HLH，无一例死亡。
我们研究的独特之处在于存在富含中性粒细胞的真皮和皮下浸润，与结缔组织病（CTD）和链球菌感染相关。我们的研究表明，EBV阴性的CHP预后往往比先前的研究更好，填补了中国人群中CHP急需的详细信息空白。此外，CHP可能在合并原发性疾病时表现为一种反应性过程；需要进一步研究来验证这些发现。
