大疱性系统性红斑狼疮合并狼疮肾炎一例

患者,女性,29岁。主因脱发2年,水肿伴疱疹1个月余就诊。血常规正常,尿常规RBC 6-10个/HP,24小时尿蛋白定量4.29 g,血白蛋白19.9 g/L。自身抗体ANA1:10000（S）,抗ds-DNA(+),抗Sm（+）,抗nRNP(+)。IgG 21.8g/L,补体C3：0.292 g/L,C4:0.037 g/L。确诊为系统性红斑狼疮伴狼疮肾炎,皮损组织病理检查：表皮下水疱形成,真皮乳头明显嗜中性细胞浸润,真皮中层血管周围灶状淋巴细胞浸润。免疫组化：皮肤直接免疫荧光法见基底膜带IgG阳性。诊断：大疱性系统性红斑狼。予甲强龙1 mg/Kg,联合环磷酰胺及硫酸羟氯喹治疗1周,患者狼疮性肾炎好转,但疱疹仍加重。予甲强龙冲击250mg×3天,加用盐酸米诺环素后好转。     

大疱性系统性红斑狼疮一例

患者,女,27岁。咳嗽4个月,躯干、四肢红斑、水疱及脱发2个月。皮肤科情况:头顶发稀疏,面部充血性红斑,双手指腹红斑,躯干四肢可见大小不等水疱、血疱,疱壁紧张,尼氏征阴性。外周血红细胞和血红蛋白下降、补体下降,ANA、抗ds-DNA阳性,血清抗VII胶原抗体阳性。患者疱液免疫检测:ANA、抗ds-DNA、抗SSA、抗SSB、抗组蛋白抗体(+),抗RO 52抗体(+++),抗核小体抗体(++),滴度与血液中滴度平行。诊断为大疱性系统性红斑狼疮。     

大疱性系统性红斑狼疮二例

2例患者均为女性,年龄分别为36岁和19岁。临床表现均为红斑基础上的水疱,尼氏征阴性。皮肤组织病理检查示：表皮角化不全,有中性粒细胞浸润,基底细胞液化变性。直接免疫荧光示IgA、lgG和C3呈线状或带状沉积于基底膜带。临床表现及实验室检查均符合系统性红斑狼疮的诊断标准。均给予糖皮质激素治疗后好转。     

线状盘状红斑狼疮伴皮肤钙沉着症1例

报告1例线状盘状红斑狼疮伴皮肤钙沉着症。患者女,36岁。因左侧头皮,额部,颧部和颊部带状萎缩性红斑20余年,局部起白色小结节2年就诊。皮肤科检查见左侧头皮额部,颧部和颊部有一纵行的带状萎缩性红斑,表面和毛细血管扩张和粘着性鳞屑,在额部萎缩性红斑上可见一半粒绿豆大的白色小结节,质硬,实验室检查示血钙和血磷均正常。分别取萎缩性红斑和白色小结节行组织病理检查,符合盘状红斑狼疮和皮肤钙沉着症组织病理学改变。     

误诊为系统性红斑狼疮的皮肤型Rosai-Dorfman-Destombes病一例

患者,女,60岁。面部对称性红斑1年,加重伴丘疹、斑块形成6个月。皮肤组织病理检查示：皮肤真皮深层及皮下组织弥漫性淋巴浆细胞浸润,组织细胞增生可见“伸入运动”,免疫组化S-100、CD68阳性,CD1a阴性,给予泼尼松、甲氨蝶呤、沙利度胺治疗2个月后好转。     

Follicular lupus erythematosus: a new cutaneous manifestation of systemic lupus erythematosus

Summary We report the clinical, histopathological and immunological features of follicular erythema and petechiae in a 30-year-old Japanese woman with systemic lupus erythematosus (SLE). Histology showed this eruption to constitute a cutaneous manifestation of SLE. To our knowledge, this is the first reported case of follicular erythema and petechiae in association with SLE. Accordingly, we propose that this rare eruption be termed 'follicular lupus erythematosus'.     

系统性红斑狼疮165例临床分析

目的：探讨165例SLE住院患者的临床特点。方法：对165例SLE患者的临床资料进行了回顾性分析。结果：肾损害总发生率最高达78.5%。少年组蝶形红斑、肾损害、脱发等发生率较高,病情进展快,2例发生狼疮危象。老年组起病不典型,自身抗体阳性率较低。男女患者临床表现及免疫学特征无显著差异,但男性患者内脏受累较重。结论：本院165例SLE住院患者,肾损害发生率高,少年组病情进展快,老年组起病不典型,男性组症状较重。     

大疱性系统性红斑狼疮临床资料分析

分析国内近20年发表的大疱性系统性红斑狼疮38篇文献,共57例,其中女47例,男10例,平均(26.6±11.8)岁;典型皮损为暗红色斑疹或正常皮肤上出现水疱大疱;常累及血液系统(64.9%)、肾脏(61.4%)及关节(57.9%);ANA阳性率为100%、dsDNA阳性率为66.7%、Sm抗体阳性率为59.6%;89.1%患者症状得到控制,其中23.6%患者单用糖皮质激素有效,76.4%患者联用DDS或其他免疫抑制剂。     

系统性红斑狼疮并发横贯性脊髓炎

报告1例系统性红斑狼疮(SLE)并发横贯性脊髓炎.患者女,23岁.患SLE 5个月,突然出现双下肢乏力、感觉缺失、排便困难3 d就诊.体格检查示脐以下皮肤感觉障碍明显,双侧膝、腱反射( ),Babinski征( ),Chaddock征( ).脊髓磁共振成像(MRI)检查可见颈3～胸12水平脊髓内片状高信号.给予甲泼尼龙冲击治疗有效.     

Systemic lupus erythematosus complicated with protein-losing enteropathy: a case report and review of the published works

A 45-year-old man was referred to our hospital with a history of multiple erythematous skin lesions of several months' duration. Blood examination revealed extreme hypoproteinemia and hypoalbuminemia, as well as the presence of antinuclear antibodies. A skin biopsy specimen showed liquefaction degeneration at the dermoepidermal junction and dense lymphocyte and neutrophil infiltration around the vessels and appendages in the upper and middle dermis. Chest X-ray and computed tomography showed a pleural effusion and thoracic paracentesis revealed a mononuclear cell-dominant cell infiltration, suggestive of serositis. Technetium-99m ((99m)Tc)-labeled human serum albumin scintigraphy and α(1)-antitrypsin clearance revealed protein leakage along the digestive tracts from the stomach to the jejunum. From the above findings, the patient was diagnosed with systemic lupus erythematosus (SLE) complicated by protein-losing enteropathy (PLE). Treatment with oral prednisolone significantly improved his clinical symptoms and hypoalbuminemia. This case highlighted the utility of (99m)Tc-labeled human serum albumin scintigraphy and α(1)-antitrypsin clearance in the diagnosis of PLE. We also present a published work review on PLE associated with connective tissue disease revealing a relatively higher prevalence in patients of Asian ethnicity, including Japanese.     

Sweet's syndrome associated with systemic lupus erythematosus: A case report and review of the literature

A 35‐year‐old Japanese female patient with systemic lupus erythematosus (SLE) presented with fever, erythematous papules and nodules, and polyarthralgia. Skin biopsy of a nodule was compatible with Sweet's syndrome. The papules/nodules were well treated with an oral glucocorticoid. Thirty cases of Sweet's syndrome associated with lupus erythematosus (LE) have been reported in the published work. The mean age was 34.2 years. They showed a higher male ratio (male : female, 1:2) compared with patients with SLE (1:9) and Sweet's syndrome (1:3.7). Sweet's syndrome may occur as a manifestation of LE, and a moderate dose of an oral glucocorticoid will result in a good response.     

大疱性系统性红斑狼疮3例及文献复习

报告3例大疱性系统性红斑狼疮(BsLE).3例患者均为女性,年龄分别为36、57和23岁.临床上均表现为全身泛发水疱、大疱.皮损组织病理检查显示为表皮下水疱,真皮乳头层较多中性粒细胞浸润,2例出现基底细胞液化变性.直接免疫荧光检查:2例示免疫球蛋白G(IgG)和(或)免疫球蛋白A(IgA)、免疫球蛋白M(IgM)抗体线状沉积于基膜带(BMZ),1例IgA线状沉积于BMZ,无lgG、IgM沉积.3例均符合SLE的诊断标准.糖皮质激素和氨苯砜可有效控制水疱、大疱的发生.单独以IgA介导的BSLE在临床上罕见.     

HLA-DM基因多态性与SLE相关性分析

目的：探讨HLA-DM基因的多态性及其与系统性红斑狼疮（systemic lupus erythematosus ,SLE）的相关性。方法：应用限制性片段长度多态性-聚合酶链反应（RFLP-PCR）技术,检测HLA-DM基因的多态性,继而分析HLA-DM基因多态性与SLE发生的相关性。结果：①共检出HLA-DMA等位基因型4种,基因型6种,HLA-DMB等位基因型3种,基因型6种,HLA-DM单倍型29种。②HLA-DM*0101/0103基因型的分布在SLE组高于健康对照组（P<0.05）,余各等位基因型及基因型在两组间的分布差异无显著性。结论：HLA-DM基因多态性与SLE的发生有一定的相关性,HLA-DM*0101/0103基因型可能为SLE的易感基因型。     

The circulating lymphocyte profiles in patients with discoid lupus erythematosus and systemic lupus erythematosus suggest a pathogenetic relationship

BACKGROUND: Discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) are chronic inflammatory diseases of unknown aetiology; the relationship of DLE with SLE has been a subject of debate for many years. OBJECTIVES; To find evidence for systemic immune activation in DLE by analysis of the immunophenotypic profiles of circulating lymphocytes, and to compare these changes with those in patients with SLE. METHODS: The immunophenotypic profile of peripheral blood lymphocyte subsets from 23 DLE patients without clinical or laboratory evidence of systemic disease, 25 SLE patients and 38 healthy donors was characterized by two-colour immunofluorescence flow cytometry analysis. None of the patients was receiving corticosteroid or immunosuppressive treatment. RESULTS: Patients with DLE had increased numbers of circulating HLA-DR+ CD3+ T cells and HLA-DR+ CD4+ T cells, indicating systemic T-cell activation, and an expansion of CD5+ CD19+ B cells. Decreased numbers of T-cell subsets expressing the differentiation markers CD11b and CD16/56, and of CD16/56+ natural killer cells were also found. In SLE, the changes were similar but more pronounced. In addition, a profound CD4+ T-cell lymphopenia and an increase of HLA-DR+ CD8+ T cells were found only in SLE. CONCLUSIONS: Our data provide evidence for systemic activation of the cellular immune system in patients with purely cutaneous DLE. Similarities in the lymphocyte immunophenotypic profiles in patients with DLE compared with SLE suggest that there are common immunopathological processes in these two conditions.     

Recent advances in cytokines in cutaneous and systemic lupus erythematosus

Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous‐limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous‐limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor‐α and interleukin‐18 to LE, especially SLE and CLE.     

167例系统性红斑狼疮患者的临床及胸部影像学分析

目的：分析系统性红斑狼疮(SLE)患者的临床及胸部影像学改变，提高对本病的认识：方法：分析了167例确诊为SLE患者的临床及胸部X线平片改变．包括26例患者的CT片分析。结果：167例患者中胸部X线异常者89例(53．3％)，主要表现为双肺、心脏、胸膜和膈肌的改变。其中两种或两种以上改变并俘者65例(38．9％)。26例胸部CT检查均显示异常。部分影像学改变早于临床表现。结论：XLE的胸部X线及CT检查对早期胸部病变的诊断有参考价值，但由于相对缺乏特异性，需结合临床及实验室检查综合判断。     

Atypical fibroxanthoma in a young female patient with systemic lupus erythematosus: A case report and literature review

A 23-year-old woman presented with an asymptomatic erythematous subcutaneous nodule on her right shin that had been present for the preceding 6 months. She had been diagnosed with systemic lupus erythematosus (SLE) and Sjogren's syndrome 6 years ago and was controlled with prednisolone and hydroxychloroquine. The nodule was excised and the pathologic diagnosis was atypical fibroxanthoma. This case is reported because this tumor occurred in an atypical site in a young female patient with SLE. The occurrence of this unusual atypical fibroxanthoma is believed to be related to altered immunity.     

Cutaneous lupus erythematosus and its association with systemic lupus erythematosus: A nationwide population-based cohort study in Korea

Although lupus erythematosus is known to be more common among women of color, the study populations in previous reports were predominantly Caucasian and there is scarce information on Asian patients. Therefore, we performed a retrospective study using a nationwide population-based cohort in South Korea. The average annual incidence of cutaneous lupus was 4.36/100 000. Among 634 patients with cutaneous lupus, 20.8% had systemic disease: cutaneous lupus was diagnosed before systemic lupus in 4.26% and after systemic lupus in 8.52%. More female patients than male patients developed systemic lupus erythematosus. The average time to progression to systemic lupus was 1.53 ± 1.46 years.     

Pancreatic panniculitis in active systemic lupus erythematosus

This report documents the case of a 64‐year‐old African‐American female with new end‐stage renal disease (ESRD), diagnosed with systemic lupus erythematosus (SLE) on renal biopsy and serologies including a positive ANA (>1:2560), positive anti‐Sm antibodies, low titer anti‐RNP antibodies, high titer anti‐Ro antibodies, anti‐dsDNA antibodies, lupus anticoagulant, and hypocomplementemia. She was also noted to have tender nodules on the bilateral shins. Excisional biopsy of one of the nodules showed marked fat necrosis with “ghost cells” and patchy basophilic granular debris consistent with pancreatic panniculitis. Further examination for pancreatic pathology showed an elevated lipase of 585 U/L (reference range 8‐78 U/L) and amylase of 214 U/L (reference range 25‐125 U/L). However, computed tomography imaging showed no evidence of pancreatitis or pancreatic tumors. This is very similar to another case recently reported in the literature. Similarities of these two cases (African‐American females with lupus nephritis on dialysis) may represent a particular subset of SLE patients at increased risk for pancreatic panniculitis.