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Amyloid deposition is frequently observed in skin lesions of hypertrophic lupus erythematosus.

Four cases of Hypertrophic Lupus Erythematosus (HLE) were reported. The lesions of HLE were observed on the forearms, face and hands in all four cases. Clinically, the lesions were erythematous, hyperkeratotic plaques. The clinical course was marked by chronicity and progression of the lesion. Histologically, marked hyperkeratosis, parakeratosis, acanthosis, degenerative changes of basal cells in H/E stain, and thickened, multilayered basement membrane in PAS stain, were observed. The observations of Dylon stain revealed that localized amyloid deposition was observed in all four cases of HLE lesions, as fluorescent-orange colored amyloid deposits in the papillary dermis and subepidermal areas at near orjust below the dermo-epidermal junction appeared under fluorescent microscope. On the basis of clinical and histological observations, we suggest that chronic irritation, such as sunlight exposure over a long-duration, might have caused the characteristic abnormalities at the dermo-epidermal junction and also initiated the frequency of amyloid deposits locally secondary to the diseases. We compared our HLE cases to other types of lupus erythematosus (LE) skin lesions, as to whether deposition of amyloid materials were frequently observed or not. Amyloid deposition was observed in one case of DLE and none of the SLE cases. Localized amyloid deposition was more frequently observed in skin lesions, secondary to HLE disease, as compared to other types of LE.

Aged↗

Lupus erythematosus in children: a report of six cases.

The clinical features of childhood discoid lupus erythematosus (DLE) are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high levels of transition to systemic disease. Systemic lupus erythematosus (SLE) is the most common rheumatic disease associated with significant morbidity and mortality in children. This is a retrospective study reporting all cases of childhood lupus erythematosus observed in the dermatology department of Habib Thameur Hospital over a 14-year period. From 1989 to 2003, six cases of childhood lupus erythematosus are included, three patients with discoid lupus erythematosus (2 girls, 1 boy), and three patients with systemic lupus erythematosus (2 boys, 1 girl). The mean age of onset was 12 years (range 10-16 years). Skin manifestations were localized in sun exposed areas in both discoid and systemic lupus erythematosus. Photosensitivity was noted in all cases. The diagnosis was confirmed by histopathologic examination, direct immunofluorescence, and immunologic findings. Treatment included sun avoidance, oral hydroxychloroquine, and topical and systemic steroids. An average follow-up time was 18.1 months (1-96 months). The severity of onset of SLE is usually greater in children than adults. We note that lupus erythematosus is not a static disease and progression from DLE to SLE is possible.

Adolescent↗

Anti-C1q autoantibodies amplify pathogenic complement activation in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) often develop glomerulonephritis (i.e., inflammation in the glomeruli of the kidney), commonly referred to as lupus nephritis. Patients with lupus nephritis typically have autoantibodies to the complement classical pathway protein C1q. Whether these anti-C1q antibodies play any role in the development of lupus nephritis has been unclear. In this issue of the JCI, a new study demonstrates that anti-C1q antibodies can amplify glomerular injury but only when they are bound within the glomerulus to C1q that has been already brought to that site by other types of glomerular-reactive autoantibodies. These studies are the first, to our knowledge, to provide a causal link between anti-C1q antibodies and target organ damage in SLE.

Animals↗

Epidemiology and socioeconomic impact of skin disease in lupus erythematosus.

The prevalence rates of systemic lupus erythematosus (SLE) may vary within 17-48/100,000 population worldwide. Although population-based epidemiological studies are still missing, the cutaneous variants of lupus erythematosus (LE) are 2-3 times more frequent than SLE itself. The most common age of onset is 20-40 y. Overall, cutaneous LE is regarded as a variant with less severe course and better prognosis. However, CDLE and SCLE last for many years and may lead, like SLE, to severe disability for work and limited life quality; also, a small proportion of patients with cutaneous LE develops SLE during the course of their disease. This implies considerable amount of medical management and costs for the community. Early recognition of cutaneous LE patients at risk to develop SLE and preventive measures against disease triggering factors are important tasks for physicians attending with cutaneous LE patients. It seems that signs of nephropathy, elevated ANA-titers and arthralgias may serve as prognostic predictors for transition into SLE. Characteristic features of cutaneous LE are photosensitivity and female predominance. UV light is a major environmental triggering factor in cutaneous LE. Skin lesions may be induced or preexistent lesions may exacerbate due to UV light in up to 80-90% of all patients. Therefore, socioeconomic counseling of the young patients, for example choice of occupation and sun protection, are essentials in compliant patients. Also, since females are 3-6 times more frequently affected than males, the possibility of hormonal influences including pregnancy and estrogen-containing drugs should be discussed. Risk considerations for females wishing to become pregnant are required, and avoidance of estrogen-containing contraceptives should be recommended.

Adolescent↗

Cardiac abnormalities in patients with systemic lupus erythematosus.

OBJECTIVE: To determine the prevalence of cardiac abnormalities in patients with systemic lupus erythematosus. DESIGN: Prospective survey. SETTING: Rheumatic diseases unit of a university hospital. PATIENTS: Volunteer sample comprising 83% of patients with systemic lupus erythematosus followed annually in the rheumatic disease unit (93 patients; mean age 46 +/- 13 years; female 79, male 14). These patients were age-matched with 16 female control volunteers (mean age 43 +/- 5 years) recruited from hospital staff. INTERVENTIONS: Electrocardiograms, two-dimensional echocardiograms and radionuclide angiograms were performed in patients and controls. Anticardiolipin antibodies were measured by enzyme-linked immunosorbent assay in the systemic lupus erythematosus patients. MAIN RESULTS: At least one cardiac abnormality was detected in 44 of 93 systemic lupus erythematosus patients (47%). These abnormalities included: aortic valve thickening 12%; mitral valve thickening, prolapse, vegetations or stenosis 23%; left ventricular segmental dysfunction 4%; left ventricular global hypokinesis 4%; right ventricular hypokinesis 4%; left ventricular hypertrophy 14%; left ventricular diastolic dysfunction 16%; and pericardial effusion 2%. Three of the 16 controls (19%) had cardiac abnormalities consisting of mitral valve prolapse (one), right ventricular hypokinesis (one) and pericardial effusion (one). Cardiac abnormalities were more common in the systemic lupus erythematosus group compared with controls (47% versus 19%, P less than 0.05). Raised anticardiolipin antibodies were specific (88%) but not sensitive (33%) for the presence of cardiac abnormalities in systemic lupus erythematosus patients. Renal disease and prednisone therapy were more common in systemic lupus erythematosus patients with cardiac involvement than in such patients without evidence of cardiac disease (40% versus 16%, P = 0.03; and 81% versus 59%, P = 0.04, respectively). CONCLUSIONS: Cardiac abnormalities can be identified noninvasively in 47% of patients with systemic lupus erythematosus.

Adult↗

Cutaneous lupus erythematosus during the neonatal and childhood periods.

Cutaneous lupus erythematosus may be related to maternal autoantibody production in the neonatal lupus syndrome, or may occur later in childhood, with or without findings of systemic lupus erythematosus (SLE). In the first section, we will discuss the transient, passively transferred neonatal disease, and in the second, we will discuss the persistent, actively acquired forms of lupus erythematosus in childhood.

Child↗

Immune-complex deposition in the eye in systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) and lupus retinopathy showed resolution of subretinal edema documented with fluorescein angiography. Subsequently at autopsy, immunofluorescence studies disclosed ocular deposition of immunoglobulins in the vascular layer of choroid capillaries and basement membranes of ciliary processes and bulbar conjunctivas. To our knowledge, these findings represent the first reported documentation of probable immune-complex ocular vasculitis in lupus retinopathy using immunofluorescent techniques, and they support the hypothesis that lupus retinopathy is caused by immune complex deposition as are other manifestations of SLE.

Adolescent↗

Levamisole in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus complicated with lupus nephritis were treated with levamisole, an immunomodulator. Clinical features, including urinary protein excretion and creatine clearance, were restored in one patient, also immunoparameters such as ANA, Ig-bearing cell number and PHA-skin test. The other patient did not respond to levamisole but did respond to cyclophosphamide.

Adolescent↗

Pregnancy in patients with systemic lupus erythematosus.

This retrospective study of 31 patients with systemic lupus erythematosus during 38 pregnancies shows a spontaneous or missed abortion rate of 7.9%, elective abortion rate of 10.5%, and a perinatal mortality rate of 12.9%. There was one maternal death 5 weeks post partum. If the onset of systemic lupus erythematosus during pregnancy included nephritis or significant thrombocytopenia, the mothers were acutely ill. All of the perinatal mortality occurred in these patients. Management of systemic lupus erythematosus during pregnancy need not differ from that in the nonpregnant state. However, immunosuppressive therapy should not be diminished or discontinued during pregnancy. Clinical parameters, renal function studies, and hematologic information were far more useful than immunologic laboratory data in assessing the course of systemic lupus erythematosus during pregnancy and indicating alterations in treatment. Antepartum fetal surveillance is advised. The timing of and route of delivery must be individualized, and systemic lupus erythematosus in and of itself is not an indication for delivery by cesarean section.

Abortion, Missed↗

The history of lupus erythematosus.

The history of lupus can be divided into the classical period which saw the description of the cutaneous disorder, the neoclassical period which saw the description of the systemic or disseminated manifestations of lupus, and the modern period which was heralded by the discovery of the lupus erythematosus cell in 1948.

Europe↗

Clinical relevance of antibodies to Ro/SS-A and La/SS-B in subacute cutaneous lupus erythematosus and related conditions.

Ro/SS-A autoantibodies are frequently associated with subacute cutaneous lupus erythematosus, neonatal lupus erythematosus and Sjögren's syndrome. The Ro/SS-A autoantigen is a ribonucleoprotein complex consisting of at least four protein components and four small cytoplasmic RNA components designated hY RNA 1, 3, 4 and 5. Three of the Ro/SS-A peptides have been isolated and cloned. The function of this ribonucleoprotein complex is as yet unknown.

Autoantibodies↗

[Erythermalgia and systemic lupus erythematosus].

We report three cases of erythermalgia associated with systemic lupus erythematosus corresponding to different clinical situations in such an association. The first patient developed erythermalgia during the course of systemic lupus erythematosus. In the second, erythermalgia preceded other symptoms of systemic lupus erythematosus by four years. In the third, erythromelalgia was not related to a flare-up of systemic lupus erythematosus, but to thrombocythemia, a complication of immunosuppressive therapy. These cases permit a discussion on terminology and classification of erythromelalgia and erythermalgia. However, more than terminology or classification into three types or into adult-onset and early-onset (childhood) erythromelalgia, the important is to consider primary and secondary forms. We used a classification into two types: primary (or erythermalgia) with subdivision into sporadic and familial subtypes, and secondary with subdivision into erythermalgia related to myeloproliferative disorders and erythermalgia related to other diseases, such as systemic lupus erythematosus, or to drugs (erythermalgia-like syndrome).

Adolescent↗

[Lupus erythematosus].

Our paper summarizes the clinical and photobiological studies on lupus erythematosus (LE) performed at the Department of Dermatology, University of Düsseldorf, during the last 5 years. A total of 190 LE cases were examined and classified according to the various cutaneous forms of LE. Except for lupus erythematosus tumidus (LET), we found a female predominance in all other groups. Discoid lupus erythematosus (DLE) is the most common variant of cutaneous LE. Subacute cutaneous lupus erythematosus (SCLE), a less common type, is characterized by the presence of Ro-antibodies, although this marker does not correlate with the degree of photosensitivity. The rare LET is an extremely photosensitive form of LE, which clearly prefers the male sex.

Adolescent↗

Rapid onset of massive ascites as the initial presentation of systemic lupus erythematosus.

Ascites in systemic lupus erythematosus (SLE) is rarely massive, and either accompanies the typical manifestations of active disease or results from nephrotic syndrome, protein-losing enteropathy, constrictive pericarditis, and conditions unrelated to lupus. Marked ascites has been attributed to chronic lupus peritonitis, characterized by the insidious onset of massive, painless ascites and unrelated to disease activity. Regardless of the etiology, ascites typically has a gradual onset and occurs after a diagnosis of SLE has been made. We describe a young woman presenting with the rapid development of massive ascites as the initial manifestation of SLE.

Adult↗

Immune dysregulation accelerates atherosclerosis and modulates plaque composition in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) have accelerated atherosclerosis. The underlying mechanisms are poorly understood, and investigations have been hampered by the absence of animal models that reflect the human condition of generalized atherosclerosis and lupus. We addressed this problem by transferring lupus susceptibility to low-density lipoprotein (LDL) receptor-deficient (LDLr-/-) mice, an established model of atherosclerosis, creating radiation chimeras with NZM2410-derived, lupus-susceptible, B6.Sle1.2.3 congenic or C57BL/6 control donors (LDLr.Sle and LDLr.B6, respectively). LDLr.Sle mice developed a lupus-like disease characterized by production of double-stranded DNA autoantibodies and renal disease. When fed a Western-type diet, LDLr.Sle chimeras had increased mortality and atherosclerotic lesions. The plaques of LDLr.Sle mice were highly inflammatory and contained more CD3+ T cells than controls. LDLr.Sle mice also had increased activation of CD4+ T and B cells and significantly higher antibody to oxidized LDL and cardiolipin. Collectively, these studies demonstrate that the lupus-susceptible immune system enhances atherogenesis and modulates plaque composition.

Animals↗

Central serous chorioretinopathy in patients with systemic lupus erythematosus.

PURPOSE: To describe three patients with systemic lupus erythematosus in whom ophthalmoscopic and fluorescein angiographic evidence of central serous chorioretinopathy developed. METHODS: The authors retrospectively reviewed the clinical and photographic records of three patients with systemic lupus erythematous in whom central serous chorioretinopathy developed. RESULTS: Ophthalmoscopic changes observed in these patients with systemic lupus erythematosus included discrete areas of clumping and mottling of the retinal pigment epithelium (RPE), focal RPE detachments, serous elevations of the neurosensory retina, and late subretinal fibrosis with scar formation. Fluorescein angiographic findings included transmission hypofluorescence and hyperfluorescence corresponding to focal RPE alterations, early punctate intense hyperfluorescence corresponding to RPE leaks with progressive filling of sub-RPE detachment spaces, and slow late filing of subretinal detachment spaces. CONCLUSION: Patients with systemic lupus erythematosus are at increased risk to have central serous chorioretinopathy develop. The pathogenetic implications for an association between systemic lupus erythematosus and central serous chorioretinopathy as well as the similarity to the chorioretinopathy seen with accelerated hypertension, pregnancy, hemodialysis, organ transplantation, and exogenous and endogenous hypercortisolism are discussed. Focal choroidal vasculature compromise with secondary dysfunction of overlying RPE cells is the proposed common mechanism.

Adult↗