Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patients

Background Lupus erythematosus (LE) is a chronic, autoimmune disease resulting from an interaction of genetic, environmental and hormonal factors and characterized by a spectrum of clinical forms with variable evolution from a localized cutaneous form to a life‐threatening systemic form. Objective To analyse and compare the prevalence and characteristics of the main clinical and immunological manifestations of subacute cutaneous LE (SCLE) and chronic CLE (CCLE). Methods A total of 270 patients with CLE (112 patients with SCLE and 158 patients with CCLE) were studied retrospectively. The clinical and serological characteristics of all the patients were collected in a chart review. Results The patients with SCLE had a higher prevalence of annular and papulosquamous lesions, Raynaud phenomenon, mucous membrane ulcers, malar rashes, photosensitivity, vasculitis and a lower frequency of discoid lesions and alopecia compared with patients with CCLE. Patients with SCLE had a higher prevalence of arthralgias (P < 0·001), xerophthalmia (P = 0·045), arthritis (P < 0·001), nephropathy (P = 0·003) and systemic LE (SLE) (P < 0·001) compared with patients with CCLE. Patients with SCLE also had a higher frequency of laboratory and serological abnormalities than patients with CCLE. Generalized discoid LE (DLE) was associated with a higher prevalence of photosensitivity (P < 0·001), panniculitis (P = 0·009) and SLE (P = 0·003) than localized DLE. In patients with SCLE and those with CCLE, photosensitivity, arthralgias, arthritis, nephropathy and xerophthalmia were associated with SLE. In patients with SCLE, significant correlations existed between clinical and immunological data. Conclusions In our series, differences in the expression of CCLE and SCLE were found with respect to the distribution and type of lesions, systemic features and immunological findings.     

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'.     

Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus

Background  Lupus erythematosus tumidus (LET) is a rare disease which was first described in 1909 but has not always been considered as a separate entity of cutaneous lupus erythematosus (CLE) in the international literature.
Objectives  To compare characteristic features of different subtypes of CLE and to analyse whether LET can be distinguished as a separate entity in the classification system of the disease.
Methods  The study involved 44 patients with CLE, including 24 patients with LET, 12 with discoid lupus erythematosus (DLE) and eight with subacute CLE (SCLE), from two centres in Germany. A core set questionnaire and an SPSS database were designed to enable a consistent statistical analysis.
Results  Location of skin lesions did not differ significantly between the CLE subtypes; however, the activity score was significantly lower in LET than in DLE ( P  <   0·01), and the damage score was significantly lower in LET than in SCLE ( P  <   0·01) and DLE ( P  <   0·01). Photosensitivity and antinuclear antibodies were confirmed to be different in LET compared with SCLE and DLE but without statistical significance. Moreover, histological analysis of skin biopsy specimens showed that abundant mucin deposition is significantly more present in LET compared with SCLE ( P  <   0·01) and DLE ( P  <   0·01) while prominent interface dermatitis and alteration of hair follicles were absent in LET.
Conclusions  Several significant differences were found between LET and other subtypes of CLE with regard to clinical, histological and laboratory parameters. These data strongly indicate that LET should be defined as a separate entity in the classification of CLE.     

沙利度胺治疗不同类型红斑狼疮皮损的疗效观察

目的：观察沙利度胺(thalidomide)对不同类型红斑狼疮(LE)皮肤损害的临床疗效。方法：选择56例不同类型的IE患者，包括慢性皮肤型红斑狼疮(CCLE)19例，深部红斑狼疮(LEP)2例，红斑狼疮-扁平苔藓综合征(LE-LP)2例，亚急性皮肤型红斑狼疮(SCLE)7例和系统性红斑狼疮(SLE)26例。患者口服沙利度胺，初始剂量为100—150mg／d，2周后根据疗效反应酌情减量，最后维持量为12．5—25mg／d；疗程6～8个月。结果：各型IE患者的皮肤损害均对沙利度胺有良好反应，56例中有41例皮损痊愈，近期治愈率达73．2％，复发率为21．9％。结论：沙利度胺对各型LE皮肤损害有较好的疗效。     

Epidemiology of cutaneous lupus erythematosus in a tertiary referral centre in Singapore

The aim of this retrospective study was to investigate the epidemiology, yield of investigations and proportion of patients who develop systemic lupus erythematosus (SLE) among the subsets of cutaneous lupus erythematosus (LE) in the Singapore Asian population. One hundred and twenty-five patients were diagnosed with cutaneous LE on clinico-pathological correlation, of which 73 had discoid lupus erythematosus (DLE), eight had subacute cutaneous LE (SCLE), 22 had acute LE lesions and the remainder had other less common forms of cutaneous LE. Histology was consistent with LE in 94.4% and suggestive in 4.8%. Direct immunofluorescence was positive in 61% of DLE, 86% of SCLE and 80% of acute LE cases. Antinuclear antibody (ANA) was present in the majority of acute LE (85%) and SCLE (88%) but only in 25% of DLE. Eight patients (11%) presenting with DLE had definite SLE at first presentation and two (2.7%) subsequently several months later. Of these patients, six had only mucocutaneous and serological criteria but two had major organ involvement. Five SCLE patients (63%) fulfilled the criteria for SLE, including two with major organ involvement.     

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.     

狼疮性肾炎患者肾组织FaS和Bax抗原的表达

为探讨Fas,Bax抗原的表达与狼疮性肾炎（lupus nephritis,LN)发病过程之间的关系，用免疫组化方法检测狼疮肾活检标本石蜡切片中Fas,Bax抗原的表达情况，狼疮性肾炎Bax抗原阳性表达率为70％，肾小管，肾小球，间质三个部位均有阳性表达，Fas抗原阳性表达率为75％，阳性表达仅见于肾小管，Fas,Bax阳性表达与LN病理分型间无明显的相关性（P＞0．05），促凋亡基因的过度表达，可能是狼疮性肾炎的发生，发展的重要因素。     

Paraneoplastic subacute cutaneous lupus erythematosus associated with cholangiocarcinoma

Subacute cutaneous lupus erythematosus (SCLE) is a dermatosis that occurs in genetically predisposed individuals. The exogenous stimulus that triggers this condition is usually unknown; however, medication is often implicated. Malignancy is a rare cause. We present a case of paraneoplastic SCLE to cholangiocarcinoma and briefly review the features of this interesting entity.     

Extracoporeal photochemotherapy in cutaneous lupus erythematosus

We report two female subjects with therapy-resistant cutaneous lupus erythematosus (LE), one with subacute cutaneous and the other chronic discoid LE, both treated with extracorporeal photochemotherapy (ECP). The responses after six and eight cycles of ECP led to a prolonged remission of 18 and 11 months, respectively. ECP seems to be a treatment option for patients not responding to or showing unwanted side-effects during conventional standard therapy.     

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。     

13例新生儿红斑狼疮的临床特征、皮损后遗表现

【摘要】 目的 探讨新生儿红斑狼疮（NLE）的皮损后遗表现，分析可能的相关因素。方法 回顾2016—2020年首都儿科研究所附属儿童医院皮肤科收集并长期随访的13例NLE，分析患儿及母亲临床特征、患儿皮损后遗表现。结果 13例皮损发病时间为出生后0 ~ 120 d，平均15 d，随访时间15 ~ 43个月，皮损形态主要为环状红斑、斑丘疹、鳞屑，皮损消退时间2 ~ 18个月（平均7.4个月）。6例皮损消退后出现色素异常表现，3例同时出现色素减退和色素沉着，2例仅有色素减退，1例仅有色素沉着，1例色素减退患儿在随访18个月后色素减退复色，未见毛细血管扩张以及萎缩或瘢痕。8例患儿母亲孕前无异常，4例患儿母亲抗ANA、抗SSA/Ro、抗SSB/La抗体均阳性。结论 NLE的皮肤受累可出现后遗皮肤表现，常见为色素异常，多数长期未消退，应注意患儿母亲可能存在的潜在性免疫系统异常。     

Linear childhood cutaneous lupus erythematosus following blaschko lines

We report a case of a 3-year-old Spanish boy with linear cutaneous lupus erythematosus following the lines of Blaschko on his face and neck. Histological examination established the diagnosis. Only eight cases of discoid lupus erythematosus following the lines of Blaschko have been reported previously.     

Pathogenesis of subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus (SCLE) is a photosensitive form of lupus‐specific skin lesion that is strongly associated with the presence of anti‐Ro/SSA autoantibody. The pathogenesis of SCLE includes genetic, environmental and immunologic factors. Recent studies provide strong evidence for the involvement of innate and cell‐mediated immunity, underlying the important role of plasmacytoid dendritic cells, interferon‐α and antibody‐dependent cell cytotoxicity. In addition, a variety of cytokines, chemokines and adhesion molecules have been found to participate in the expansion phase of the autoimmune effector mechanisms. This article summarizes the recent immunological findings and reviews the current mechanisms which are implied in the development of the disease.