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混合性脂膜炎经环孢素 A 治疗 12 个月后缓解:病例报告。

Mixed panniculitis responding to cyclosporin-A with a 12-month follow-up: a case report.

出版信息

Int J Immunopathol Pharmacol. 2009 Oct-Dec;22(4):1143-6. doi: 10.1177/039463200902200433.

DOI:10.1177/039463200902200433
PMID:20074481
Abstract

Panniculitides represent a heterogeneous group of inflammatory diseases involving subcutaneous fat. Subcutaneous fat is normally organized into adipose cells, adipocytes, and septa of connective tissue. The inflammation involving such tissues can be more represented in septa (septal panniculitis) or in lobules (lobular panniculitis) or be equally distributed in both (mixed panniculitis). A bioptical study is necessary in order to discern between different forms. Vascular involvement is also different in such diseases, as it can interest arteries, or veins, or both. Different grades of fat necrosis can also be observed, such as adipocytes without nuclei, lipophagic necrosis, liquefactive fat necrosis, microcystic fat necrosis, ischaemic fat necrosis. Panniculitis can be idiopathic or secondary to other diseases such as systemic sclerosis, rheumatoid arthritis, systemic erithematous lupus and many others. Therapies usually vary on the single patient but the general orientation leads to the use of immunosuppressive drugs such as thalidomide, corticosteroids, cyclosporin-A, hydroxychloroquine and cyclophosphamide. We report a case of a 19-year-old female affected by primary mixed panniculitis, associated with fever and deep asthenia, that resolved in a few weeks and was maintained with oral cyclosporin-A.

摘要

脂膜炎是一组累及皮下脂肪的异质性炎症性疾病。皮下脂肪通常由脂肪细胞、脂肪细胞和结缔组织隔组成。累及这些组织的炎症可以更集中在隔(间隔性脂膜炎)或小叶(小叶性脂膜炎),或在两者之间均匀分布(混合性脂膜炎)。为了区分不同类型,需要进行组织学研究。此类疾病中的血管受累也不同,它可以影响动脉、静脉或两者都影响。也可以观察到不同程度的脂肪坏死,如无核脂肪细胞、噬脂性坏死、液化性脂肪坏死、微囊状脂肪坏死、缺血性脂肪坏死。脂膜炎可以是特发性的,也可以是继发于其他疾病,如系统性硬皮病、类风湿关节炎、系统性红斑狼疮等。治疗方法通常因个体而异,但一般的治疗方向是使用免疫抑制剂,如沙利度胺、皮质类固醇、环孢素 A、羟氯喹和环磷酰胺。我们报告了一例 19 岁女性患有原发性混合性脂膜炎,伴有发热和深度乏力,在数周内缓解,并通过口服环孢素 A 维持。

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