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。     

Morphea with discoid lupus erythematosus

The presence of lupus erythematosus with morphea in the same patient has rarely been reported. In this case, we describe a woman with the overlap of discoid lupus erythematosus with superficial morphea, diagnoses that are supported by histopathologic features and laboratory studies.     

A Study of lupus erythematosus with particular reference to generalized discoid lupus

The classification of lupus erythematosus (LE) is difficult because of variable and multisystem involvement, occurrence of transitional forms, and overlapping with connective tissue disorders. One of the earliest attempts at classification was made by O'Leary (1934), who classified LE clinically into four main types: (1) chronic discoid lupus erythematosus (DLE), or fixed type with the typical erythematous, scaly, well-demarcated eruption, usually showing follicular plugging and atrophy, confined to the head region; (2) generalized DLE, or chronic disseminated type differing from the localized discoid type in that the erythematous plaques are found not only on the head area but also below the neck region (O'Leary considered that constitutional systems were generally lacking in these two types); (3) subacute disseminated LE; and (4) acute disseminated LE. The last two categories reflect what we now call systemic lupus erythematosus (SLE) of varying severity. Many authors have used O'Leary's clinical classification, but progress in laboratory medicine—in particular the discovery of the LE cell—has led to other schemes, the most widely accepted at present being that related to the American Rheumatism Association's (ARA) preliminary criteria for the diagnosis of SLE (Cohen et al., 1971). These criteria have been criticized by many authorities because they do not include important data, such as those relative to the antinuclear antibody test, serum anti-native-DNA, serum complement levels, many neurological abnormalities, renal biopsy changes, and cutaneous immunofluorescent (IF) findings. However, Dubois (1974b) pointed out that all categorizations of LE are arbitrary, and he accepted the ARA criteria as a successful method for differentiating LE. This report involves the clinical and laboratory findings in 123 patients with LE seen at the Mayo Clinic during the 3-year period 1971–1974 and an assessment of the validity and usefulness of O'Leary's classification.     

Systemic lupus erythematosus with generalized chronic cutaneous (discoid) lupus erythematosus

A 24-year-old pregnant African-American woman had a 3-4 year history of chronic, scarring, hyperpigmented plaques on her scalp, face, trunk, and extremities. She complained of joint pain and fatigue. Clinical presentation, laboratory data, and histopathologic features were consistent with systemic lupus erythematosus in a patient with generalized chronic discoid lupus erythematosus. This subtype is a distinct lupus erythematosus subset that rarely develops renal disease and has a relatively benign but chronic course.     

Multiple dermatofibromas associated with lupus profundus

We report a 58-year-old Afro-Caribbean woman who presented with more than 20 dermatofibromas on the body particularly on the back, arms and legs. These developed spontaneously over the course of 5 years. She also had a long-standing 5 x 2-cm area of lipoatrophy on the right upper arm and a 2-year history of several inflammatory subcutaneous nodules developing on the upper chest and left breast. These were confirmed histologically as lupus profundus. Apart from a mild arthritis, she had no other markers for systemic lupus erythematosus and was systemically well. Multiple dermatofibromas are rare. There are around 30 reports of multiple dermatofibromas associated with systemic diseases. More than half of these cases were associated with systemic lupus erythematosus, with or without systemic steroid therapy and about one-third were associated with HIV infection. Although the mechanism is unknown, it appears that multiple dermatofibromas are associated with autoimmune diseases or altered immune states. This is the first case of multiple dermatofibromas associated with lupus profundus. The knowledge of such associations may contribute to the understanding of the pathogenesis of dermatofibromas, which is as yet unknown.     

Discoid lupus erythematosus with secondary amyloidosis

BACKGROUND: Secondary localized cutaneous amyloidosis is a clinically unapparent phenomenon associated with various cutaneous pathologies, usually tumours of epidermal origin. The amyloid is thought to be derived from keratinocytes. OBJECTIVES: To characterize the amyloid deposition observed incidentally within lesional biopsies from three patients with discoid lupus erythematosus (DLE), and retrospectively to study the phenomenon within DLE skin samples. METHODS: Localized amyloid deposition was observed in three cases of DLE by immunofluorescence studies, and these cases were further studied by histology and immunohistochemistry using a monoclonal anticytokeratin antibody. Retrospective histological review of DLE tissue specimens archived over 12 months was performed to look for evidence of previously undetected amyloid. RESULTS: Amyloid deposition was confirmed histologically in the three index cases by staining with Congo red and thioflavin T. Positive staining with an anticytokeratin antibody demonstrated the epidermal origin of the amyloid protein. Of the 18 archived cases reviewed amyloid was retrospectively detected in one sample. CONCLUSIONS: Secondary cutaneous amyloidosis of keratinocyte origin can be seen in DLE lesions. It may be a not infrequent occurrence and may remain under-reported. We discuss the possible role of disease chronicity and colloid body degradation in the pathogenesis of amyloidosis.     

Three siblings with systemic lupus erythematosus

We present the cases of three siblings with systemic lupus erythematosus (SLE). The diagnosis was made when the sisters were of age 21, 25 and 28 years. They shared some clinical features, including typical facial rash, photosensitivity and Raynaud's phenomenon, and tested positive for antinuclear antibodies. However, their symptoms and clinical courses varied. Human leukocyte antigen (HLA) typing revealed that DR4 and A2 were present in all three sisters, while HLA type A11, B35 and B54 were each found in two of the three sisters. The two elder sisters developed lupus glomerulonephritis 8 and 11 years after the onset of SLE. It is suggested that there are genes responsible for the onset of the disease and also unknown regulatory genes other than HLA result in different phenotypes.     

33例SLE患者狼疮带试验与临床资料分析

目的：探讨LBT临床意义及在SLE诊断标准中的价值。方法：收集我科1996年6月～2002年7月间住院病人33例对LBT检查结果及临床资料进行分析。结果：33例中24例LBT阳性，总阳性率72．7％(24／33)，其中24例皮损、7例暴光部位正常皮肤、2例非暴光部位正常皮肤LBT阳性率分别为75％(18／24)、57．1％(4／7)、100％(2／2)。Ig沉积种类及ANA、anti—dsDNA、C3异常者与病情活动有关。结论：对不满足ARA标准的病人，LBT是诊断SLE指标之一。     

Discoid lupus erythematosus treated with cryotherapy

A female is presented with multiple longstanding hypertrophic discoid lupus erythematosus lesions resistant to topical and systemic therapy for years. She suffered severe itching and pain localized to the lesions. Treatment with cryosurgery resulted in complete healing of the lesions, leaving slightly hypopigmented soft scars. There was no tendency of relapse during a follow-up period of ten years.