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PubMed1992

[Deep lupus erythematosus in children].

Deep cutaneous lesions are seldom encountered in lupus erythematosus. The disease described in the literature as lupus erythematosus profundus or lupus erythematosus panniculitis usually occurs in middle-aged women. The authors report a case of deep lupus erythematosus which was exceptional in that it occurred in an 11-year old girl. The lesions were situated on the face and consisted of solid subcutaneous nodules and clear-cut ulcerations leaving atrophic pigmented scars. The histological image of a nodule was one of lobular lymphocytic panniculitis with homogeneous hyalinization of adipose nodules, to which must be added periadnexal and perivascular dermo-epidermal lymphocytic infiltrates. Granular IgM deposits arranged along the dermo-epidermal junction were observed at direct cutaneous immunofluorescence. Laboratory examinations showed leucopenia (3,300/mm3) with neutropenia (1,100/mm3) and the presence of antinuclear antibodies at 1/100 speckled fluorescence, as well as antibodies directed against native DNA. Studies of renal function and for complement gave normal results. The other causes of lobular panniculitis were excluded. The lesions regressed within 3 weeks under hydroxychloroquine; this drug was also successful in arresting a relapse consecutive to withdrawal of treatment. The authors have analysed the 17 paediatric cases of deep lupus erythematosus and were able to determine their main characteristics: 1. The lesions occur mostly in girls (70 p. 100). They are located electively on the face and the lateral aspect of the shoulders. They consist of well-limited, solid or hard subcutaneous nodule which may congregate to form plaques. The epidermis may be normal or pathological, poikilodermic, looking like a discoid or ulcerated lupus erythematosus. The lesions regress, leaving a characteristic atrophic scar.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Antinuclear↗
PubMed2002

Management of skin disease in patients with lupus erythematosus.

Skin disease in patients with lupus erythematosus may be subdivided into two broad categories - those represented by a 'specific' histopathology, the interface dermatitis, and those with changes that are not specific to lupus erythematosus, for example, vasculitis, mucin infiltration, etc. The specific skin lesions that are most common are discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). Evaluation will allow the treating physician to assign a prognosis. Cutaneous lesions can generally be managed with standard therapies. Patients with discoid LE and subacute cutaneous LE are generally photosensitive, and therefore sunscreens, protective clothing and behavioural alteration should be discussed with all patients. Topical corticosteroids are a standard form of therapy, but 'newer' agents such as retinoids, calcipotriene and tacrolimus might be effective. Antimalarial agents are generally effective. Attempts to reduce or stop smoking may aid in the control of cutaneous LE. The choice of alternative therapy is personal, and discussions of the risks and benefits should be carefully documented.

Adult↗
PubMed1976

Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus are described who in the course of their disease developed small atrophie blanche lesions that closely resembled those found in malignant atrophic papulosis. Preliminary investigation of these two cases indicates that considerable similarities probably exist in the pathogenesis of malignant atrophic papulosis and the atropie blanche lesions of systemic lupus erythematosus. It is concluded that a diagnosis of malignant atrophic papulosis should only be made after systemic lupus erythematosus has been excluded by full investigation.

Adult↗
PubMed2002

Systemic lupus erythematosus in childhood.

Systemic lupus erythematosus is a disease of immune dysregulation that strikes approximately 1 in 2000 individuals. The usual patient is a young woman of child-bearing age; however, this illness affects patients of all ages, ethnic backgrounds, and both sexes. Twenty percent of all cases of lupus are diagnosed during the first two decades of life. Perhaps the most essential point in treating a child with lupus is to be aware and concerned about how to deliver treatment to a patient in the middle of their physical, intellectual, and emotional development.

Adolescent↗
PubMed2006

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.

Combined Modality Therapy↗
PubMed1998

Therapeutic advances in systemic lupus erythematosus.

In treating systemic lupus erythematosus, clinicians need to consider not only the organ involvement and the complications of therapy, but also associated conditions such as premature atherosclerosis and thrombosis. A variety of agents are now available to treat cutaneous disease, including the antimalarials and thalidomide. Controversy exists about the most appropriate immunosuppressive regimen in severe disease. Experience with using cyclosporine, mycophenolate mofetil, dehydroepiandrosterone, and intravenous immunoglobulin is increasing, but plasmapheresis has not been shown to be of benefit. Autologous bone marrow transplantation has been performed. The advent of the biological era, particularly with monoclonal antibodies, gives promise of more targeted therapy.

Arteriosclerosis↗
PubMedDate not supplied

[Chronic lupus erythematosus presenting as acneiform lesions].

BACKGROUND: Cutaneous manifestations of lupus erythematosus are numerous but usually permit easy diagnosis. However, there are atypical lesions that can mimic benign dermatologic disorders. We report on a patient with lesions of acne leading to the diagnosis of chronic lupus erythematosus, and who subsequently developed systemic lupus erythematosus. CASE REPORT: A 30-year-old woman presented with inflammatory lesions and comedos on the face. The eruption started after her last pregnancy and was refractory to local and general treatment. She also complained of arthralgia, Raynaud's phenomenon and diffuse alopecia. Cutaneous biopsy was characteristic of chronic lupus erythematosus. Immunofluorescence microscopy of lesional skin showed a lupus band deposit. Antinuclear antibodies were highly positive. The patient was successfully treated with chloroquine. Three years later, the patient presented with photodistributed eruption. Antinuclear antibodies were still positive and in addition anticardiolipin antibodies were found. Final diagnosis was systemic lupus erythematosus. DISCUSSION: Acneiform lesions are rarely reported in lupus erythematosus. Only three similar cases were reported in literature. Atypical and treatment-resistant eruptions should attract attention. Furthermore, the occurrence of systemic lupus in chronic lupus erythematosus is not an unfrequent phenomenon and the oestrogen-dependance of chronic lupus lesions may predict this association.

Acneiform Eruptions↗
PubMedDate not supplied

Lupus erythematosus panniculitis in a patient with autoimmune hepatitis.

Lupus erythematosus panniculitis (profundus), a rare variant of chronic panniculitis, sometimes develops during the course of discoid lupus erythematosus or systemic lupus erythematosus. A 61-year-old woman had suffered from autoimmune hepatitis type I for 5 years. Prednisolone had been administered as maintenance therapy and her hepatitis had been well controlled. However, asymptomatic erythematous indurated nodules developed symmetrically in both pre-auricular regions, and skin biopsy revealed lupus erythematosus panniculitis (profundus). Increase in dosage of prednisolone resolved the skin lesion, leaving depressed atrophic scars. This is the first report of lupus erythematosus panniculitis complicating autoimmune hepatitis.

Biopsy↗
PubMed2002

Prevalence of systemic lupus erythematosus in central Saudi Arabia.

OBJECTIVE: Although systemic lupus erythematosus of varying severity has been seen in our clinics, there is no information regarding the prevalence of the disease among Saudi nationals. We conducted this study to determine the prevalence of systemic lupus erythematosus in Al-Qaseem region of the Kingdom of Saudi Arabia. METHODS: A community survey of towns and villages in the Al-Qaseem area of central Saudi Arabia was conducted in 3 phases to determine the prevalence of systemic lupus erythematosus in the region. RESULTS: Of the 10,372 studied, 2 cases of systemic lupus erythematosus were identified using the criteria set for the diagnosis of systemic lupus erythematosus by the American College of Rheumatology. Based on that, the prevalence of systemic lupus erythematosus was estimated to be 19.28 per 100,000 population in the region. CONCLUSION: The estimated prevalence of systemic lupus erythematosus in Al-Qaseem area is similar to that found in western countries.

Adolescent↗
PubMedDate not supplied

Systemic lupus erythematosus presenting with pericardial tamponade and lupus pneumonitis.

A previously-well 37-year-old woman presented with pericardial tamponade and pulmonary infiltrates. Systemic lupus erythematosus was diagnosed. Pericardiocentesis and systemic treatment with prednisolone produced dramatic improvement with no recurrence. The patient underwent bronchoalveolar lavage and transbronchial lung biopsy, which established the diagnosis of lupus pneumonitis.

Adult↗
PubMed2000

Racial group, socioeconomic status, and the development of persistent proteinuria in systemic lupus erythematosus.

OBJECTIVES: Systemic lupus erythematosus (SLE) patients of Afro-Caribbean and Asian origin living in the United Kingdom have a more severe spectrum of disease compared with the white population but whether this is attributable to genetic host factors or environmental factors is unclear. This study examines time from first symptom to onset of persistent proteinuria, as a marker of renal disease, to assess which factors are important. METHODS: The 189 patients studied were ascertained using multiple methods and included 161 white, 22 Afro-Caribbean and six Asian patients. Time of first observation of persistent proteinuria (>/=0.5 g/day) was taken as onset of renal SLE. Initial univariate analysis to determine which factors are associated with onset of renal disease was followed by using a Cox's proportional hazards regression model enabling analysis of several prognostic factors at the same time. Variables included three measures of socioeconomic status, ethnic group and the presence or absence of different autoantibodies. RESULTS: There was no effect from any socioeconomic variable. Using forwards stepwise selection, the following had independent effects (p<0.05) on the development of renal SLE: Afro-Caribbean race (hazard rate ratio 4.4 (1.9-10.2), compared with white population); and the presence of IgG anti-cardiolipin antibodies (hazard rate ratio 2.6 (1.2-5.7)). CONCLUSION: Differing socioeconomic factors do not explain the increased frequency of lupus nephritis in Afro-Caribbean patients with SLE, but rather there are important genetic or other host differences. The independent effect of IgG anti-cardiolipin antibodies warrants further investigation.

Adolescent↗
PubMed2000

Alterations of cerebral glucose metabolism indicate progress to severe morphological brain lesions in neuropsychiatric systemic lupus erythematosus.

Neuropsychiatric systemic lupus erythematosus (SLE) is frequently associated with deficits in brain glucose metabolism, even if morphological imaging by magnetic resonance imaging (MRI) shows no abnormalities. In these patients it is unclear whether or not the changes of brain metabolism measured by F-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) may progress to lesions of cerebral structure. We describe a 20-year-old woman with SLE who presented with depression, headache and impairment of memory. Initially, a cranial MRI was negative, but FDG-PET revealed significant hypometabolism in the frontal and parieto-temporo-occipital regions on both sides as well as hypermetabolism in the nuclei caudati. Within two months the patient developed an acute confusional state, seizures, visual disturbances and cranial MRI became positive showing hyperintensities at the basal ganglia and the temporo-occipital regions. Focal cerebral symptoms responded to treatment with high dose corticosteroids and brain lesions in MRI disappeared. However, a second FDG-PET showed persistent hypometabolism at frontal regions in accordance with the persistence of subclinical depression. To our knowledge, this is the first SLE case report showing that functional brain lesions visualized by FDG-PET may be a risk factor for subsequent structural brain damage seen in MRI. Thus, FDG-PET may help to verify cerebral involvement of SLE earlier than MRI.

Adult↗
PubMed2004

Defining response in systemic lupus erythematosus: a study by the Systemic Lupus International Collaborating Clinics group.

OBJECTIVE: In a preliminary attempt to develop a drug responder index for patients with systemic lupus erythematosus (SLE), 2 validated disease activity instruments were studied for their responsiveness and compared to a physician visual analog scale (VAS) assessment of disease activity. We attempted to determine whether these validated instruments were useful components in characterizing response in the setting of a clinical trial. METHODS: Eighty paper patients were assessed using the British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Disease Activity Index (SLEDAI) and by physician's assessment of global activity. The cases were arranged in random order and divided into groups of 20 patients and each group was assessed by 20 lupus experts; change in disease activity was recorded at 3 and 6 months compared to baseline using a physician VAS. RESULTS: Four different lupus experts assessed disease activity in all 80 patients at baseline and 3 and 6 months after initiation of therapy using the BILAG and SLEDAI instruments. BILAG and SLEDAI scores correlated well over time; however, in a regression analysis where average physician VAS were chosen as the outcome variable, a significant amount of variation in the average physician VAS not related to the SLEDAI and BILAG scores was noted. CONCLUSION: The physician VAS may be too blunt to assess response in SLE, because even among experienced lupus assessors, there were considerable differences in what influenced scoring decisions.

Adult↗
PubMed1990

Experimental reproduction of skin lesions in lupus erythematosus by UVA and UVB radiation.

Sunlight is a well-established factor in the induction and exacerbation of lupus erythematosus. Although experimental reproduction of lupus erythematosus lesions with wavelengths shorter than 320 nm was demonstrated previously, the effect of wavelengths longer than 320 nm was not investigated adequately. In this study we show that the action spectrum of lupus erythematosus reaches into the UVA region. A total of 128 patients with lupus erythematosus underwent phototesting with the use of polychromatic UVB and long-wave UVA. Subsets of the disease consisted of discoid lupus erythematosus (n = 86), subacute cutaneous lupus erythematosus (n = 22), and systemic lupus erythematosus (n = 20). Skin lesions clinically and histologically compatible with lupus erythematosus were induced in 64% of patients with subacute cutaneous lupus erythematosus, 42% of patients with discoid lupus erythematosus, and 25% of patients with systemic lupus erythematosus. The action spectrum of the induced lesions was within the UVB range in 33% of patients, in the UVA range in 14%, and in the UVB and UVA range in 53%. In positive test reactions patchy dark erythema and urticarial plaques developed within a few days. In some patients typical discoid lesions persisted for months.

Adolescent↗
PubMed2006

Familial chilblain lupus, a monogenic form of cutaneous lupus erythematosus, maps to chromosome 3p.

Systemic lupus erythematosus is a prototypic autoimmune disease. Apart from rare monogenic deficiencies of complement factors, where lupuslike disease may occur in association with other autoimmune diseases or high susceptibility to bacterial infections, its etiology is multifactorial in nature. Cutaneous findings are a hallmark of the disease and manifest either alone or in association with internal-organ disease. We describe a novel genodermatosis characterized by painful bluish-red inflammatory papular or nodular lesions in acral locations such as fingers, toes, nose, cheeks, and ears. The lesions sometimes appear plaquelike and tend to ulcerate. Manifestation usually begins in early childhood and is precipitated by cold and wet exposure. Apart from arthralgias, there is no evidence for internal-organ disease or an increased susceptibility to infection. Histological findings include a deep inflammatory infiltrate with perivascular distribution and granular deposits of immunoglobulins and complement along the basement membrane. Some affected individuals show antinuclear antibodies or immune complex formation, whereas cryoglobulins or cold agglutinins are absent. Thus, the findings are consistent with chilblain lupus, a rare form of cutaneous lupus erythematosus. Investigation of a large German kindred with 18 affected members suggests a highly penetrant trait with autosomal dominant inheritance. By single-nucleotide-polymorphism-based genomewide linkage analysis, the locus was mapped to chromosome 3p. Haplotype analysis defined the locus to a 13.8-cM interval with a LOD score of 5.04. This is the first description of a monogenic form of cutaneous lupus erythematosus. Identification of the gene responsible for familial chilblain lupus may shed light on the pathogenesis of common forms of connective-tissue disease such as systemic lupus erythematosus.

Adolescent↗
PubMed1994

Depressive symptoms in patients with systemic lupus erythematosus: association with central nervous system lupus and Sjögren's syndrome.

OBJECTIVE: To determine if patients with systemic lupus erythematosus (SLE) with depressive symptoms differ in regard to organ involvement and serological activity from other patients with SLE. METHODS: Disease manifestations were compared between 71 patients with SLE with a history of depressive symptoms and 278 patients without a history of depressive symptoms by univariate analysis and multiple logistic regression. RESULTS: Both univariate and logistic regression analysis revealed an association of depressive symptoms with neuropsychiatric lupus and secondary Sjögren's syndrome (SS). Patients with neuropsychiatric lupus had an adjusted odds ratio of 3.43 (95% CI 2.55, 4.63; p = 0.00005), and patients with secondary SS had an adjusted odds ratio of 2.97 (95% CI 2.08, 4.25; p = 0.0006) for depressive symptoms. No other organ involvement or serological abnormality was associated with depressed mood. CONCLUSION: These discrete associations of depressive symptoms with neuropsychiatric lupus and secondary SS suggest that depression does not occur purely as a response to social stresses, and may be a manifestation of autoimmune disease in some patients.

Adult↗
PubMed1988

Myocardial involvement in systemic lupus erythematosus detected by magnetic resonance imaging.

Myocardial involvement in systemic lupus erythematosus is commonly found at autopsy but seldom recognized clinically or by routine cardiological investigations. As the magnetic resonance relaxation parameter, T1, is altered by changes in tissue cellularity, we carried out magnetic resonance imaging in 10 patients with systemic lupus erythematosus. Five had active systemic lupus erythematosus when assessed using the lupus activity criteria count. The mean (+/- SD) T1 was 319 +/- 12 in normal volunteers and 321 +/- 10 in a second control group with hypertrophic cardiomyopathy. In the group with systemic lupus erythematosus, there was a higher mean value of 336 ms with a wider scatter of individual results (SD +/- 22 ms). In the subgroup of patients with active disease, T1 was significantly higher (349 +/- 24) than in either of the two control groups. In addition, there was an inverse correlation between serum complement and myocardial T1 in patients with systemic lupus erythematosus. Myocardial abnormalities in systemic lupus erythematosus were demonstrated by magnetic resonance imaging even where other non-invasive cardiac investigations were negative. We conclude that T1 calculated from magnetic resonance imaging is often abnormal in systemic lupus erythematosus and probably indicates myocardial involvement.

Adult↗
PubMed1988

A patient with systemic lupus erythematosus presenting both central nervous system lupus and steroid induced psychosis.

We describe a patient with systemic lupus erythematosus (SLE) who presented with both steroid induced psychosis and central nervous system (CNS) SLE at different times. The cerebrospinal fluid (CSF) immunoglobulin indices and electroencephalogram were found to be helpful and reliable for the correct diagnosis of both conditions. There have been no reports of patients with SLE who showed both steroid induced psychosis and CNS SLE, and our patient has provided substantial evidence that both conditions can occur in the same individual.

Adult↗