Cassis Tamella B, Fearneyhough Paul K, Callen Jeffrey P
Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, 310 E. Broadway, Louisville, KY 40202, USA.
J Am Acad Dermatol. 2004 Mar;50(3):465-9. doi: 10.1016/s0190-9622(03)02784-1.
Lupus erythematosus (LE) panniculitis (LEP) is a form of chronic cutaneous LE most often characterized by erythematous subcutaneous nodules. The histopathology of LEP may be distinctive, allowing a diagnosis even in the absence of any other LE features. Lymphocytic infiltration of the panniculus is termed lymphocytic lobular panniculitis. This entity has been reported in both LEP and subcutaneous panniculitis-like T-cell lymphoma. We describe a 67-year-old woman who presented with multiple dermal and subcutaneous nodules on her legs and hips. Biopsy specimens of roughly 15 lesions were interpreted as being diagnostic of LEP, however, with each subsequent biopsy specimen more atypical cells were observed. The patient responded only to moderate doses of oral prednisone, and failed intralesional triamcinolone acetonide, oral immunosuppressive agents, oral antimalarial agents, and oral thalidomide. Three years after the onset of her disease, ulcerated plaques and nodules developed. At this time a T-cell receptor gene rearrangement was present and subcutaneous panniculitis-like T-cell lymphoma was diagnosed. Despite multiple doses of chemotherapy she died approximately 1 year after diagnosis. Patients with apparent LEP that have atypical lymphocytes in their biopsy specimens should be followed up closely for the development of T-cell malignancy.
红斑狼疮（LE）脂膜炎（LEP）是慢性皮肤型红斑狼疮的一种形式，最常见的特征是皮下出现红斑性结节。LEP的组织病理学表现可能具有特异性，即使在没有任何其他红斑狼疮特征的情况下也能做出诊断。脂肪层的淋巴细胞浸润称为淋巴细胞性小叶性脂膜炎。这种情况在LEP和皮下脂膜炎样T细胞淋巴瘤中均有报道。我们描述了一名67岁女性，其腿部和臀部出现多个皮肤和皮下结节。约15个病变的活检标本被诊断为LEP，然而，随后的每个活检标本中都观察到更多非典型细胞。该患者仅对中等剂量的口服泼尼松有反应，而病灶内注射曲安奈德、口服免疫抑制剂、口服抗疟药和口服沙利度胺均无效。疾病发作三年后，出现了溃疡斑块和结节。此时存在T细胞受体基因重排，诊断为皮下脂膜炎样T细胞淋巴瘤。尽管进行了多次化疗，她在诊断后约1年死亡。活检标本中有非典型淋巴细胞的疑似LEP患者应密切随访，观察是否会发展为T细胞恶性肿瘤。
