Merelli M, Quartuccio L, Bassetti M, Pecori D, Gandolfo S, Avellini C, De Vita S
Infectious Disease Clinic, University Hospital Santa Maria della Misericordia, Udine, Italy.
Rheumatology Clinic, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy.
Clin Exp Rheumatol. 2015 Nov-Dec;33(6):906-9. Epub 2015 Nov 17.
Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis characterized by systemic features, due to histiocytic infiltration along with haemophagocytosis, which may also appear in bone marrow, spleen, lymph nodes, and liver. Haemophagocytic lymphohistiocytosis (HLH) is a group of autoinflammatory disorders, which include macrophage activation syndrome, sometimes observed in the course of systemic autoimmune diseases, such as juvenile chronic polyarthritis, systemic lupus erythematosus or vasculitis, and infection-associated haemophagocytic syndrome; if not promptly recognised and treated, HLH can be fatal. Visceral leishmaniasis (VL) is a systemic disease caused by different forms of Leishmania spp., an intracellular protozoa. VL is endemic in tropical countries such as in the Middle East and the Mediterranean. The typical clinical and laboratory features are fever, hepato-splenomegaly, hypergammaglobulinaemia and pancytopenia. The features of VL may mimic some haematologic diseases. We report a case of cytophagic histiocytic panniculitis and HLH, triggered by a previous visceral leishmania infection. Cyclosporine was quickly effective in this case, after failure of high-dose glucocorticoids, anakinra and etoposide.
噬血细胞性组织细胞性脂膜炎（CHP）是一种罕见的脂膜炎，其特征为具有全身症状，这是由于组织细胞浸润并伴有噬血现象，这种情况也可能出现在骨髓、脾脏、淋巴结和肝脏中。噬血细胞性淋巴组织细胞增生症（HLH）是一组自身炎症性疾病，其中包括巨噬细胞活化综合征，有时在系统性自身免疫性疾病过程中观察到，如青少年慢性多关节炎、系统性红斑狼疮或血管炎，以及感染相关噬血细胞综合征；如果不能及时识别和治疗，HLH可能会致命。内脏利什曼病（VL）是由不同形式的利什曼原虫属（一种细胞内原生动物）引起的全身性疾病。VL在中东和地中海等热带国家流行。典型的临床和实验室特征为发热、肝脾肿大、高球蛋白血症和全血细胞减少。VL的特征可能与某些血液系统疾病相似。我们报告一例由先前的内脏利什曼病感染引发的噬血细胞性组织细胞性脂膜炎和HLH病例。在大剂量糖皮质激素、阿那白滞素和依托泊苷治疗失败后，环孢素在该病例中迅速起效。
