Nailfold capillary abnormalities in patients with Sjögren's syndrome and systemic lupus erythematosus

It is unknown whether primary Sjögren's syndrome (pSS) and systemic lupus erythematosus (SLE) have the same nailfold capillary abnormality, although they show similarity in several clinical. immunological and genetic aspects. Microscopy of the nailfold capillaries was undertaken in the two diseases and the observed parameters were compared with age- and sex-matched controls using analysis of variance. pSS and SLE showed a different frequency in their clinical and laboratory findings, except for the presence of anti-SS-A/B antibodies. All nailfold capillary parameters showed significantly higher values than those of corresponding normal controls. although between pSS and SLE there was no significant difference. The results may indicate that pSS and SLE are part of a spectrum of disorder.     

Detection of anti-basement membrane zone antibodies in bullous systemic lupus erythematosus

We describe a 42-year-old black woman with long-standing systemic lupus erythematosus in whom vesiculobullous lesions developed. Routine histologic and immunologic studies fulfilled the criteria for the diagnosis of bullous systemic lupus erythematosus. Indirect immunofluorescence showed antinuclear antibodies without basement membrane zone fluorescence. We destroyed the nuclear antigens of the indirect immunofluorescence substrate with 2 mol/L sodium chloride, which unmasked basement membrane zone linear IgG staining. We also confirmed anti-basement membrane zone antibodies by employing a new technique of direct immunofluorescence on sodium chloride-split skin. Our finding prove that a thorough search for anti-basement membrane zone antibodies can be revealing. Our results support the idea that a subset of bullous systemic lupus erythematosus has the staining characteristics of epidermolysis acquisita, with the dermal side of the split skin showing linear immunoglobulin deposition.     

Association of bullous pemphigoid with systemic lupus erythematosus

Seven previous cases of coexistent bullous pemphigoid (BP) and systemic lupus erythematosus (SLE) have been reported. An 83-year-old man had clinical, laboratory, and histologic evidence of both BP and SLE. The question of whether coexistent SLE and BP actually occur can be settled only when complete studies are available.     

Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus

Acquired ichthyosis is a condition accompanying many systemic illnesses such as lymphoma, sarcoidosis, dermatomyositis and systemic lupus erythematosus (SLE). Overlap syndromes are defined as clinical entities which satisfy each of the diagnostic criteria of two different connective tissue diseases concurrently or consecutively. The coexistence of SLE with systemic sclerosis has been very rarely reported. We describe a 33-year-old woman with an overlap syndrome consisting of systemic sclerosis and SLE who developed ichthyosis on her extremities.     

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。     

大疱性系统性红斑狼疮1例

报告1例大疱性系统性红斑狼疮，临床上符合SLE的诊断标准，并有大疱形成。组织病理改变为表皮下大疱形成，疱液中有许多嗜中性白细胞，Alcianblue染色阳性。     

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.     

大疱性系统性红斑狼疮一例

<正>临床资料患者,女,18岁。主因口腔溃疡、双上肢水肿性或荨麻疹样红斑2个月,全身泛发大疱20余天,于2009年7月31日就诊。患者2个月前无明显诱因口腔出现多个血疱伴疼痛,疱壁破溃后形成溃疡,1周后双手背、上肢出现瘙痒性红斑、风团样皮损,双眼睑肿胀,结膜充血。在当地医院以荨麻疹给予咪唑斯汀等抗过敏药物治疗后皮损有所好转。1个月前,患者背部突然出现黄豆至核桃大小水疱,自觉瘙痒,     

The immunogenetic relationship between anti-Ro(SS-A)/La(SS-B) antibody positive Sj?gren's/lupus erythematosus overlap syndrome and the neonatal lupus syndrome

We have described previously the clinical features of a unique group of anti-Ro(SS-A) antibody positive Sjogren's patients who have cutaneous features of lupus erythematosus, most commonly subacute cutaneous lupus erythematosus, defined as the Sjogren's/lupus erythematosus overlap syndrome. Three of these patients are also mothers of infants with the neonatal lupus erythematosus syndrome, characterized by cutaneous lesions resembling subacute cutaneous lupus erythematosus or congenital heart block. Patients with Sjogren's/lupus erythematosus overlap syndrome, subacute cutaneous lupus erythematosus, and mothers of infants with the neonatal lupus syndrome characteristically have autoantibodies to Ro(SS-A), and in many cases, La(SS-B) antigens. The present study was designed to test the hypothesis that anti-Ro(SS-A)/La(SS-B) positive Sjogren's/lupus overlap patients and mothers of infants with neonatal lupus erythematosus syndrome are immunogenetically homogenous and closely related. We report a strong association with HLA-B8, DR3, DQw2, and DRw52 phenotypes and the HLA-B8, DR3, DQw2, DRw52 extended haplotype in both patient cohorts. Furthermore, we describe disease associations with HLA-DR3/DRw6 heterozygotes in both patient groups. These data demonstrate that anti-Ro(SS-A)/La(SS-B) positive Sjogren's/lupus overlap patients and neonatal lupus syndrome mothers are immunogenetically closely related to each other and appear to be more closely related to both primary Sjogren's syndrome and subacute lupus erythematosus, than to classical systemic lupus erythematosus.     

Pyoderma gangrenosum in a patient with bullous systemic lupus erythematosus

We report a 55-year-old woman with bullous systemic lupus erythematosus, who later developed pyoderma gangrenosum (PG). Dapsone was effective for the eruption of bullous bullous systemic lupus erythematosus but not for pyoderma gangrenosum. Cyclosporine was effective for the skin lesions of pyoderma gangrenosum. This is the first reported case of PG associated with bullous systemic lupus erythematosus.     

儿童大疱性系统性红斑狼疮临床特点分析

目的:分析儿童大疱性系统性红斑狼疮的临床特点。方法:回顾性分析3例儿童大疱性系统性红斑狼疮资料,并总结文献报道的15例病例临床特点及治疗转归。结果:18例患儿中皮肤和肾脏病理资料完整者16例,平均年龄11.22岁,男女比例为2∶16;10例以皮肤大疱首发,12例大疱发生在狼疮肾炎之前;12例合并发热、9例合并口腔溃疡,14例出现贫血。所有病例ANA阳性,13例存在3种及以上血清自身抗体。皮肤病理特点为基底膜补体及免疫球蛋白沉积,10例24 h尿蛋白≥1.5 g; 16例患儿具有肾脏病理资料,其中Ⅴ型狼疮肾炎7例、Ⅳ型狼疮肾炎6例、Ⅲ型狼疮肾炎3例。氨苯砜对皮损有效,免疫抑制剂可减少大疱复发,利妥昔单抗和贝利尤单抗治疗对于难治性患儿可能有效。结论:多系统性受累、多种自身抗体阳性和狼疮肾炎是儿童大疱性系统性红斑狼疮的特点,免疫抑制治疗可改善预后,抗B细胞治疗可作为难治性病例的选择。     

Annular erythema associated with Sj?gren's syndrome: a variant of systemic lupus erythematosus

We present a Burmese patient with widespread annular erythema associated with Sj?gren's syndrome. Unlike previously described cases, the disease occurred in the setting of systemic lupus erythematosus. Photoprovocation testing revealed light sensitivity in the UVA range with elicitation of subacute cutaneous lupus erythematosus-like lesions. The presence of an erythema annulare centrifugum-like eruption should initiate the search for Sj?gren's syndrome and systemic lupus erythematosus.     

红斑狼疮／扁平苔藓重叠综合征一例

临床资料患者,男,66岁。主因双耳郭、手背及足跟部紫红色斑片、反复破溃伴疼痛10年余,于2012年1月13日就诊。     

A case of lupus erythematosus/lichen planus overlap syndrome

A case of lupus erythematosus/lichen planus overlap syndrome (LE/LP overlap syndrome) was reported. A 53-year-old woman developed violaceous erythema around the nostrils and the upper lips and atrophic scaly erythema on the cheeks and neck. Histopathological studies revealed that the patient had distinct discoid lupus erythematosus (DLE), LP, and a lesion with combined features of DLE and LP. Direct immunofluorescent (DIF) studies of the mixed lesion revealed both prominent immunoglobulin (Ig)G deposits in a granular pattern at the basement membrane zone (BMZ) and IgM deposits in the clusters of cytoid bodies; the former are more typical of LE and the latter more of LP. DIF features in combination were unique for LE/LP overlap syndrome. The patient was satisfactorily treated with topical tacrolimus. While reports support the effectiveness of tacrolimus in either LE or LP, this is the first case of LE/LP overlap syndrome treated with topical tacrolimus.     

Coexistence of bullous pemphigoid and systemic lupus erythematosus.

A vesiculobullous eruption with clinical and histological features of bullous pemphigoid developed in a 28-year-old woman with proven systemic lupus erythematosus (SLE). Serum of this patient contained elevated titers of antinuclear antibodies but basement membrane antibodies could not be detected at first, though they did appear in blister fluid. Normal monkey skin explants cultured on this patient's sera gave positive direct immunofluorescence (IF) at the basement membrane zone (BMZ) for IgG deposits. The use of tissue culture methods may be helpful because of the capacity of this test system to reveal the presence of the antibodies to the BMZ despite the presence of the antinuclear antibodies that appear to interfere with their demonstration in standard indirect IF tests.