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.     

Palisading granuloma presenting in a patient with systemic lupus erythematosus

A patient with systemic lupus erythematosus who developed a distinctive nodular eruption demonstrating typical features of palisading granuloma is presented herein. A 60-year-old woman was admitted complaining of an elastic-hard, indurated nodule on the right dorsal aspect of the metacarpophalangeal joint of her third finger with a history of several years. She was successfully treated for her lupus nephritis with oral predonisolone. She had experienced erythema on her cheeks after sun exposure and polyarthralgia for a decade. Laboratory examination revealed positive results for antinuclear antibody and rheumatoid factor. Peripheral blood cell counts showed leukocytopenia and lymphocytopenia. Results of hand X-ray were unremarkable. Histological examination of the skin biopsy specimen from the nodule revealed a structure composed of a central area of fibrinoid necrosis, surrounded by a middle zone of palisading cells and an outer zone of chronic lymphocyte infiltrate. These findings led us to the histological diagnosis of palisading granuloma. She was diagnosed as having systemic lupus erythematosus complicated with a rheumatoid nodule. She is currently under treatment with cyclophosphamide (50 mg/day) without exaggeration of her systemic lupus erythematosus and nodule.     

Lupus erythematosus vegetans

A 29-year-old woman with known systemic lupus erythematosus presented with exudative and vegetative plaques bilaterally on her groins. The clinical and histological findings indicated a diagnosis of pyodermatitis vegetans. Direct immunofluorescence studies revealed the presence of a definite basement membrane zone band for IgG, IgM and C3, favouring lupus erythematosus. We propose the term lupus erythematosus vegetans for this combination of clinicopathological and immunohistological findings.     

Cutaneous lupus erythematosus: a personal approach to management

SUMMARY Skin disease in patients with lupus erythematosus may be subdivided into two broad categories - those lesions that when biopsied demonstrate interface dermatitis and those that do not demonstrate interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus, subacute cutaneous lupus erythematosus and acute cutaneous lupus erythematosus. Patients with these 'specific' manifestations have varying degrees of systemic involvement from rare systemic disease in patients with localized discoid lupus erythematosus to common and often severe involvement in patients with acute cutaneous lupus erythematosus. Patients who do not demonstrate interface dermatitis also may have systemic disease and in some instances the skin manifestations are linked to some of the more severe systemic manifestations. Many patients with cutaneous lesions characterized by the interface dermatitis can be controlled with 'standard' therapies including sunscreens, protective clothing and behavioural alteration, and topical corticosteroids with or without an oral antimalarial agent. This review presents a brief summary of each common cutaneous manifestation of lupus erythematosus, its relationship to systemic involvement and treatment issues to effectively deal with the lupus erythematosus patient who has skin disease.     

Systemic lupus erythematosus with C1q deficiency

We report a case of systemic lupus erythematosus associated with C1q deficiency. Our patient presented at the age of 6 years with cutaneous lupus. She later developed Raynaud's phenomenon, non-scarring alopecia, oral ulceration and grand mal seizures due to cerebral vasculitis. Complement C3 and C4 levels were consistently normal during flares of her lupus and haemolytic activity of her complement was absent, suggesting a deficiency of an early component of the complement cascade. No C1q could be detected.     

系统性红斑狼疮的治疗进展

系统性红斑狼疮的治疗以激素和免疫抑制剂为基础。近年来,随着各相关学科的发展,新型免疫抑制剂、生物制剂、干细胞移植等相继用于治疗系统性红斑狼疮,并取得了显著疗效。此外,近年来的研究,对抗疟药、维生素D、他克莫司等在系统性红斑狼疮中的治疗作用也有了更深入的认识。本文就该方面的进展作一综述。     

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.     

Digital gangrene: a rare skin symptom in systemic lupus erythematosus.

A case of digital gangrene in a patient with systemic lupus erythematosus without secondary anti-phospholipid syndrome is reported. The acute onset of the necrotizing acrovasculitis occurred without a history of Raynaud's phenomenon. Preceding symptoms of the systemic lupus erythematosus were arthritis, photosensitivity, alopecia and anorexia. Despite the seriousness of the acute episode the patient achieved an excellent outcome.     

Coexistence of pemphigus herpetiformis and systemic lupus erythematosus

A female patient with coexistence of pemphigus herpetiformis and systemic lupus erythematosus is described. She presented to our Department with pruritic vesicles on her trunk and extremities, which were later accompanied with butterfly like erythema on her face and with central nervous system (CNS) manifestations. The diagnosis of pemphigus herpetiformis was based on the clinical picture and immunofluorescence finding, because the histopathologic finding is not always typical for the diagnosis. The diagnosis of systemic lupus erythematosus was based on positive ANA and anti-dsDNA, presence of butterfly-like erythema on her face, and CNS manifestations. The patient was treated by corticosteroids in combination with immunosuppressants, which should ensure good control of both diseases. The coexistence of pemphigus herpetiformis and systemic lupus erythematosus has not been reported in recent literature.     

A case of nodular cutaneous lupus mucinosis

The patient was a 44-year-old female. She was diagnosed as having systemic lupus erythematosus at the age of 21 years and had been receiving systemic steroid treatment (5 mg prednisolone/day). Nodules began to appear on her neck, chest and back from June in 2000, and she was referred to our clinic for examination and treatment. Examination of a biopsy sample of an eruption on the neck revealed mucin deposition in the dermis. Based on the clinical and histopathological findings, she was diagnosed as having typical nodular cutaneous lupus mucinosis. The dose of steroid was increased, and the eruptions gradually disappeared.     

结节性皮肤狼疮性黏蛋白病

报告1例结节性皮肤狼疮性黏蛋白病。患者女,42岁。患有系统性红斑狼疮,在其病程中双上肢伸侧和背部出现许多肤色或暗紫红色丘疹和结节,伴瘙痒半年就诊。皮损组织病理学检查示真皮胶原纤维束间有大量黏蛋白沉积;电镜检查示真皮成纤维细胞周围有大量无定形物质沉积。     

Discoid lupus erythematosus

Discoid lupus erythematosus is a manifestation of chronic cutaneous lupus erythematosus with a small risk of systemic involvement. In this review article, the role of predisposing factors such as haplotype, hormones, antibodies and sunlight are discussed. The clinical features, including variants and associations, and management options are presented.     

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1例

分析报告1例大疱性系统性红斑狼疮。患者以全身皮肤粘膜起红斑、水疱为表现起病，伴有口腔溃疡、双膝关节疼痛，尿蛋白3 ，ANA阳性1：160颗粒型，ds-DNA抗体阳性，ENA阳性、SS-A阳性。取躯干部皮损行组织病理活检，皿染色示：表皮下水疱，真皮浅层水肿，真皮乳头见中性粒细胞聚集，免疫病理示基底膜带可见IgG呈颗粒状沉积，符合大疱性系统性红斑狼疮病理改变。该病例为大疱性系统性红斑狼疮。     

Papulonodular mucinosis with subacute cutaneous lupus erythematosus

A 30-year-old woman with subacute cutaneous lupus erythematosus presented with a facial eruption, painful and swollen ulcers of her digits, and asymptomatic lesions on her abdomen. The laboratory findings were consistent with subacute cutaneous lupus erythematosus with an anticardiolipin antibody. A biopsy specimen of the asymptomatic lesions on her abdomen showed mucin within the upper reticular dermis. Papulonodular mucinosis is a rare but well-documented finding associated with systemic and cutaneous forms of lupus erythematosus. The etiology and pathogenesis of this finding remain to be elucidated.     

Photosensitivity in lupus erythematosus

BACKGROUND: Lupus erythematosus is a systemic disease process that may manifest with a variety of internal and cutaneous findings. Photosensitivity is one the most common manifestations of lupus erythematosus. In patients with lupus erythematosus, there is a relationship between exposure to ultraviolet light, autoantibodies, genetics and other factors in the development of photosensitivity. METHODS: Literature was reviewed on the topics of lupus erythematosus and photosensitivity discussed together and separately. The suggested mechanisms for their relationship were reviewed and analyzed. RESULTS: Photosensitivity's relationship to and influence on the systemic manifestations of lupus remain to be defined. Mechanisms for photosensitivity might include: modulation of autoantibody location, cytotoxic effects, apoptosis induction with autoantigens in apoptotic blebs, upregulation of adhesion molecules and cytokines, induction of nitric oxide synthase expression and ultraviolet-generated antigenic DNA. Tumor necrosis factor alpha also seems to play a role in the development of photosensitivity. CONCLUSION: The basis for photosensitivity in lupus has yet to be fully defined. It is more commonly associated with subacute and tumid lupus erythematosus than with other variants. Anti-Ro antibodies appear to relate to photosensitivity. Tumor necrosis factor alpha polymorphisms appear to be important in some variants of lupus with photosensitivity. There is no sine qua non antibody or mutation of photosensitivity in lupus. In patients with lupus, more work needs to be done to define the mechanisms of photosensitivity.     

The wound/burn guidelines – 4: Guidelines for the management of skin ulcers associated with connective tissue disease/vasculitis

The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.     

Anti-Ribosomal P Protein Antibodies in a Japanese Patient with Systemic Sclerosis

A 56-year-old Japanese woman developed Raynaud's phenomenon and digital ulcerations at the age of 50. She showed rapid and diffuse skin sclerosis; visceral involvement was mild. She showed no symptoms or laboratory findings suggestive of other collagen diseases, including systemic lupus erythematosus and Sjögren's syndrome. Based on the clinical and histological findings, she was diagnosed as having diffuse cutaneous systemic sclerosis (SSc). The patient's serum produced nucleolar and cytoplasmic staining by indirect immunofluorescence analyses on HEp-2 cell substrate and reacted with P0, P1 and P2 proteins in immunoblotting using purified ribosomal antigens. She was negative for anti-topoisomerase I, centromere, and U1RNP antibodies. Anti-ribosomal P protein antibodies are considered highly specific for systemic lupus erythematosus; this is the first case report of an SSc patient with anti-ribosomal P protein antibodies. The clinical features of SSc patients with these antibodies need to be clarified by an accumulation of cases.     

Cutaneous drug-induced lupus erythematosus: Clinical and immunological characteristics and update on new associated drugs

Cutaneous drug-induced lupus erythematosus (CDILE) is a lupus-like syndrome related to drug exposure which typically resolves after drug discontinuation. It can present as a systemic or a sole cutaneous form and different drugs may be associated with each form. CDILE pharmacoepidemiology is constantly changing. Indeed, older drugs primarily associated with systemic CDILE are no longer prescribed and new drugs associated with either cutaneous or systemic CDILE have emerged. The present study discusses the clinical and laboratory aspects of CDILE and the postulated pathogenesis, and it provides an update on implicated drugs. We performed a literature review to single out the new drugs associated with CDILE in the past decade (January 2010–June 2020). Among 109 drugs reported to induce CDILE in 472 patients, we identified anti-TNFα, proton-pump inhibitors, antineoplastic drugs, and, in particular, checkpoint inhibitors, as emerging drugs in CDILE. Most of the published studies are cases reports or small case series, and further larger studies as well as the development of validated classification criteria are needed to better understand and characterize their implication in CDILE.     

男性系统性红斑狼疮74例临床分析

目的：探讨男性系统性红班狼疮（SLE）的临床特点。方法：回顾性分析74例男性SLE患者的临床资料。结果：男性SLE患者的发病年龄构成相对不集中，10－20岁年龄段发病最高；易并发狼疮性肾炎且肾脏损害较重；ANA抗体、抗Sm抗体、抗ds-DNA抗体可能与狼疮性肾炎严重程度密切相关。结论：男性SLE患者在发病年龄、临床表现及特异性抗体的表达等方面有其自身特点。