Subacute Cutaneous Lupus Erythematosus Presenting in Childhood: A Case Report and Review of the Literature

A 2‐year‐old African American, Hispanic boy presented with well‐defined, violaceous, annular dermal plaques without scale over the upper extremities, face, lower extremities, and buttocks. The clinical presentation and laboratory studies were consistent with a diagnosis of subacute cutaneous lupus erythematous (SCLE). SCLE presenting in childhood is exceedingly rare, with only eight cases previously reported. It is important to clinically differentiate SCLE from other eruptions more common to children, such as atopic dermatitis, urticarial drug eruptions, and psoriasis vulgaris, because progression to systemic lupus erythematous (SLE) may occur. SLE needs to be closely followed. We present the first case (to our knowledge) of SCLE in a child of African American or Hispanic descent and provide a table of other documented pediatric presentations of SCLE for comparison.     

儿童系统性红斑狼疮18例分析

目的：了解儿童系统性红斑狼疮的临床及实验室检查特征。方法：对18例儿童系统性红斑狼疮与54例成年人患者的临床特点进行对照研究。分析其首发症状,临床表现及实验改变。结果：儿童患者肾脏损害的严重程度比成人重,其它器官损害也多于成人,但无统计学意义,未成年人组C3下降程度明显高于成年组,而CH50降低的发生率明显高于成年组,结论：系统性红斑狼疮儿童患者临床及实验室检查与成人相比存在某些程度差异。     

Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE

Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus that is often associated with autoimmunity to type VII collagen. We describe a 45-year-old woman with BSLE who presented with vesiculobullous lesions as an initial manifestation of SLE. The patient first noticed a widespread urticarial, erythematous eruption associated with tense blisters, erosions, and crusting. She was diagnosed with bullous pemphigoid and underwent a one-month course of treatment with betamethazone. Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus. The patient's IgG circulating antibodies labeled the dermal floor of salt-split skin and recognized type VII collagen in immunoblot studies. Although methylprednisolone pulse therapy for glomerulonephritis did not alleviate the vesicullobullous eruption, treatment with dapsone resulted in dramatic disappearance of the lesions. Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE. It also emphasizes the close temporal relationship between BSLE and lupus nephritis.     

An Atypical Case of Bullous Systemic Lupus Erythematosus in a 16‐Year‐Old Boy

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal blistering disease with a female predominance characterized as an acute vesicobullous eruption in patients with systemic lupus erythematosus (SLE). Here we report a case of BSLE in a 16‐year‐old boy that does not adhere to the criteria originally established and suggest a new outlook on this condition.     

Bullous Systemic Lupus Erythematosus

Two patients with bullous systemic lupus erythematosus are reported. In one, the disease appears to have been caused by hydralazine. The cutaneous lesions of bullous SLE have not been previously reported in drug-induced lupus.     

Ulcerative Colitis Associated with Preceding Systemic Lupus Erythematosus

We report a 50-year-old female patient who developed ulcerative colitis 31 after years being diagnosed with systemic lupus erythematosus. The overall clinical evaluation of her SLE activity differentiated ulcerative colitis from lupus colitis. Since the association of idiopathic systemic lupus erythematosus with ulcerative colitis has rarely been reported, the combination of these two diseases may be coincidental.     

Localized Linear Bullous Eruption of Systemic Lupus Erythematosus in a Child

Abstract: A 9-year-old girl newly diagnosed with systemic lupus erythematosus (SLE) developed a localized linear papulovesicular eruption over the right dorsal hand and ulnar forearm. The skin findings were clinically suggestive of herpes zoster, lichen striatus, or lichen planus-lupus erythematosus overlap. However, histologic, immunofluorescent, Immunoelectron microscopic, and immunobiot studies revealed findings compatible with bullous SLE. Our patient is noteworthy because she is the first one reported with bullous SLE presenting in a localized linear pattern. She is also the second-youngest reported patient with bullous SLE.     

Linear Childhood Discoid Lupus Erythematosus Following the Lines of Blaschko: A Case Report with Review of the Linear Manifestations of Lupus Erythematosus

Seventeen cases of childhood discoid lupus erythematosus (DLE) have been previously reported in the literature. We describe the first reported case of childhood linear DLE following the lines of Blaschko. The clinical and histologic characteristics of childhood DLE are discussed and a review of the linear manifestations of childhood LE is presented.     

系统性红斑狼疮1000例临床研究

本文对1962年至1992年诊治的1000例系统性红斑狼疮患者的临床资料进行分析。探讨了本病常见的诱发因素、临床表现、早期诊断及治疗与预后的关系。资料表明,32.6%的患者查无明显诱因,病情极易复发,伴严重内脏损害,预后不佳。在临床中,蛋白丢失性肠病的发生率为11.2%,应予以重视。患者3年、5年、10年、20年和30年的生存率分别为95.9%、93.5%、87.6%、66.6%和60.9%。近十年来,采用定期免疫学监测、中西医结合治疗、纤溶联合疗法治疗难治性狼疮肾炎等措施,使本病预后得以明显改善。     

Linear Childhood Discoid Lupus Erythematosus Following the Lines of Blaschko: Successfully Treated with Topical Tacrolimus

Abstract: The linear arrangement of discoid lupus erythematosus is uncommon. Here, we report a 6‐year‐old Japanese girl with linear discoid lupus erythematosus following the lines of Blaschko on her face and neck. Topical tacrolimus treatment improved the eruptions. The present case also indicated the important role of epidermal and dermal cells as well as immune cells in the pathogenesis of cutaneous lupus erythematodes.     

Lupus erythematosus panniculitis

Lupus erythematosus panniculitis is a quite rare clinical entity characterised by one or several firm, asymptomatic, often fairly large subcutaneous nodules, as a manifestation of SLE or DLE. Two cases of lupus panniculitis, both female, are described here. Both of them had absence of typical lesions of SLE or DLE elsewhere in the body. Systemic symptoms were not present, antinuclear factor was positive in one case. Both showed typical lobular panniculitis on biospy and responded favourably to systemic chloroquine therapy.     

抗核小体抗体检测在系统性红斑狼疮中的临床应用

目的探讨抗核小体抗体(AnuA)在系统性红斑狼疮(SLE)中的临床应用。方法采用酶联免疫吸附法(ELISA)检测187例SLE及84例其他风湿性疾病患者血清AnuA和抗dsDNA抗体水平,采用免疫印迹法检测抗Sm抗体。结果 AnuA诊断SLE的敏感性和特异性分别为72.19%和97.62%,其敏感性显著高于抗dsDNA抗体、抗Sm抗体(P<0.05),其特异性与抗dsDNA抗体、抗Sm抗体差异无统计学意义(P>0.05);联合检测AnuA、抗dsDNA抗体、抗Sm抗体诊断SLE的敏感性达95.72%。SLE患者AnuA阳性组与阴性组相比,阳性组患者口腔溃疡、关节疼痛发生率更高,24h尿蛋白异常和补体C3降低(P<0.05)。结论 AnuA对SLE有较高敏感性和特异性,是诊断SLE最有价值的指标之一;联合检测AnuA、抗dsDNA抗体、抗Sm抗体可提高SLE的诊断;AnuA可能与SLE疾病活动性相关。     

Pincer Nail Deformity Associated with Systemic Lupus Erythematosus

Background

Systemic lupus erythematosus (SLE) is an autoimmune disorder with various systemic and cutaneous manifestations. Nail abnormalities, including onycholysis, red lunulae, pitting, and ridging, have been seen in patients with the disorder. To our knowledge, however, pincer nail deformity has yet to be reported coincident with the onset of SLE.

Objective

We report a case of pincer nail deformity subsequent to the development of SLE and describe surgical treatment of the deformed nails. This is followed by a review of the literature.

Conclusion

Acquired pincer nail is a dystrophy with numerous reported causative associations, including psoriasis, tumors of the nail apparatus, tinea ungium, ß-blocker usage, and now SLE. Pain from the resulting constriction can interfere greatly with daily activities and can be debilitating to the extent of requiring therapy.     

皮肤型红斑狼疮的治疗现状

皮肤型红斑狼疮是红斑狼疮病谱中相对较轻的一型,治疗上有别于系统性红斑狼疮,目前尚无固定的治疗模式。局部外用糖皮质激素是广泛采用的治疗手段之一,对各种皮肤型红斑狼疮均有效。较新型的外用制剂如他克莫司及吡美莫司,主要用于治疗对糖皮质激素和常规药物无效的皮肤型红斑狼疮。对外用药物治疗无效的皮损,可选择皮损内注射糖皮质激素,以快速发挥抗炎和免疫抑制作用。细胞毒药物和沙利度胺主要治疗复发或难治性皮肤型红斑狼疮。激光及光疗也能有效改善皮肤型红斑狼疮的皮损。     

Bullous Systemic Lupus Erythematosus in a Child Responding to Dapsone

Bullous systemic lupus erythematosus is a subepidermal blistering disorder that primarily affects young women and only rarely occurs in children. We report a case of bullous systemic lupus erythematosus refractory to corticosteroid therapy in a 12‐year‐old boy who was successfully treated with oral dapsone.     

Lupus erythematosus panniculitis: a clinicopathologic study

BACKGROUND: Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus which involves the deep dermis and the subcutaneous fat. The purpose of this study was to ascertain the clinical profile of Asian patients with this condition. METHODS: This was a retrospective study of all histologically confirmed lupus panniculitis seen at our center between 1992 and 1997. The age, sex, past history/subsequent diagnosis of systemic lupus erythematosus (SLE), presence of clinical discoid lupus erythematosus (DLE) changes on overlying skin, direct immunofluorescence, serologic, and histologic findings were analyzed. RESULTS: There were 12 cases of lupus panniculitis, two of which were in patients already diagnosed with SLE and one in which the patient subsequently evolved into SLE. The mean age at diagnosis was 31.3 years. The face (50%), upper limbs (33%), and scalp (25%) were the most common sites of involvement. Thirty-three per cent had clinical evidence of DLE on the overlying skin, whilst 67% had histologic features of DLE on the overlying skin. A lupus band was present in 36%. Antinuclear antibody (ANA) was positive in three of 11 cases; these were in the two patients who already had SLE and in the only patient who progressed to SLE. All of the cases showed fat necrosis and, in the majority of cases, there was associated lobular and paraseptal inflammation. Thirty-three per cent showed lymphocytic vasculitis and 75% had mucin deposition. None had lymphoid nodules, subepidermal hyalinization, or calcification. CONCLUSIONS: Lupus panniculitis affects a younger age group in Asians as compared with the Western population. Although about one-third of patients show clinical evidence of overlying DLE, two-thirds of patients show histologic evidence of DLE. It tends to have a mild disease course in the majority of cases.     

Case of Pediatric Bullous Systemic Lupus Erythematosus Treated with Intravenous Immunoglobulin

This is a case report of a 16‐year‐old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first‐line treatment option for BSLE, but the patient's history of anemia and leukopenia and long‐term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis. She was started on intravenous immunoglobulin (IVIG), 2 g/kg divided over 3 days, with significant improvement in her cutaneous symptoms. IVIG is a treatment option for BSLE patients in whom agents such as dapsone are contraindicated.     

Lupus erythematosus panniculitis

Lupus erythematosus panniculitis is an uncommon variant of lupus erythematosus characterized by a specific involvement of the subcutaneous fat. It is a panniculitis with peculiar clinical features and histopathologically characterized by a mostly lobular panniculitis. It may appear in patients with discoid lupus erythematosus and systemic lupus erythematosus, but also as the unique manifestation of lupus erythematosus, and in the latter cases the diagnosis may be problematic. Histopathologic differential diagnosis with subcutaneous panniculitis-like T-cell lymphoma may also be extremely difficult. This article reviews the salient clinicopathologic features and treatment of lupus erythematosus panniculitis, with special emphasis on the histopathologic features.     

Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or ‘butterfly’ rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.     

Absence of human herpesvirus 8/Kaposi''s sarcoma-associated herpesvirus in a case of benign lymphangioendothelioma associated with periosteal haemangioma

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'.