[Disseminated lupus erythematosus; malignant lupus erythematosus (Goldsmith-Bear); history and concept].
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Animals↗
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Results for “LUPUS ERYTHEMATOSUS”
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[Detection of SSA/Ro antibody in patients with systemic lupus erythematosus, discoid lupus erythematosus and cutaneous photosensitivity. A comparative study].
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Antibodies, Antinuclear↗
Discoid lupus erythematosus and lupus erythematosus profundus in a child.
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Child↗
Lupus erythematosus.
Lupus erythematosus represents a wide spectrum of diseases. Within this heterogeneity, however, several clinically relevant subsets have been found that may unite differing aspects of the disease and that may have pathogenic implications for the problem as a whole. Exciting links between genetic phenotypes, certain immune responses, and related cutaneous findings in neonatal, complement-deficient, subacute cutaneous, and ANA-negative lupus erythematosus and Sjögren's syndrome have been found. The increased frequency of the anti-Ro(SSA) antibody in these groups is notable. The ultraviolet light-altered immune system found in vivo and in vitro may also be relevant to disease pathogenesis. Even if the mechanisms elaborated are not the precise aberrations in lupus erythematosus, they may serve as an operational model for further research. Continued investigations into mechanisms of photoexacerbations in lupus erythematosus, as well as into genetically mediated immune response, may further our understanding of these disorders.
Adolescent↗
Podiatric pathology of lupus erythematosus.
Lupus erythematosus is an enigmatic disease. Its podiatric manifestations are largely cutaneous, but vasculitic involvements may produce complications. The authors review much of the histopathology of lupus erythematosus that is relevant to the podiatric physician and surgeon. Awareness of the serious systemic implications of lupus erythematosus and the internal medications used in treatment is part of the physician's responsibility in understanding the whole patient.
Foot↗
Cutaneous manifestations of lupus erythematosus.
Lupus erythematosus is an autoimmune disease that demonstrates cutaneous, systemic, or both cutaneous and systemic manifestations. This article reviews the cutaneous manifestations of lupus erythematosus.
Alopecia↗
Clinical, histologic, and immunofluorescent distinctions between subacute cutaneous lupus erythematosus and discoid lupus erythematosus.
Subacute cutaneous lupus erythematosus (SCLE) was originally described and distinguished from discoid lupus erythematosus (DLE) on the basis of clinical examination of the skin, but subsequent reports have questioned the concept of SCLE as a marker of a unique subset of LE patients. We classified 27 lupus patients, on the basis of cutaneous exam, as having discoid lupus skin lesions, subacute cutaneous skin lesions, or systemic lupus erythematosus (SLE) without DLE or SCLE lesions. Clinical features most characteristic of SCLE rather than DLE were superficial, non-indurated, non-scarring lesions, and photosensitivity, with lack of induration being the single most helpful finding. Histologic examination of lesional skin showed a relatively sparse, superficial infiltrate in SCLE and a denser, deeper infiltrate in DLE. A distinctive pattern of staining with direct immunofluorescence, particulate epidermal IgG deposition, was found in seven of seven SCLE patients (all anti-Ro/SSA positive) and none of the other patients. This distinctive pattern can be reproduced experimentally when anti-Ro/SSA autoantibodies are infused into human skin-grafted mice. Particulate dermal-epidermal junctional staining was the pattern seen in the patients who did not have SCLE. Clinically defining SCLE as a superficial inflammatory form of cutaneous lupus (i.e., considering lesions to be DLE if they are indurated) results in a meaningful segregation of SCLE and DLE patient groups. The epidermal IgG deposits unique to SCLE provide independent evidence that the clinical findings that were used to identify the patient groups actually identify distinctive cutaneous lupus subsets. The observation that antibodies are present in a different location in the skin in SCLE than in DLE indicates that SCLE and DLE are likely to have different pathomechanisms.
Antibodies, Antinuclear↗
Lower extremity arterial disease in systemic lupus erythematosus.
Lupus vasculitis primarily affects microvascular circulation, and large-vessel thrombosis is a rare complication of this disease. Large-vessel occlusive disease in systemic lupus erythematosus is most likely related to hypercoagulability in addition to immune complex-mediated endothelial damage. We describe the 11th and 12th patients reported to have systemic lupus erythematosus and macrovascular occlusive disease of the lower extremities. Our experience and a review of the literature suggest that, while aortoiliac disease is amenable to bypass or endarterectomy, infrainguinal disease is rarely correctable surgically, and amputation becomes necessary in most of these patients.
Adolescent↗
Lupus tumidus: a variant of cutaneous lupus erythematosus.
Lupus tumidus is a rare sub-type of chronic cutaneous lupus erythematosus characterized by dermal plaques in which excessive mucin accumulates early in disease process. We report a middle aged women having succulent, edematous and persistent plaque over her face for five years that was not responding to various empirical treatments offered to her. Finally, on clinico-pathological basis, it was diagnosed as a case of tumid lupus erythematosus (TLE) and she responded satisfactorily to the treatment regimen including oral steroids, chloroquine and application of sun screen.
Adult↗
Cutaneous lupus erythematosus.
Lupus erythematosus (LE) has many different clinical manifestations including a variety of cutaneous findings. Some of the cutaneous manifestations are not specific for LE, such as photosensitivity reactions, oral ulcers, alopecia, urticaria, vasculitis, vesiculo-bullous lesions, acral changes, cutaneous mucinoses, and cutaneous calcinosis. Other findings are specific for LE in that they are found only in patients who have lupus erythematosus. These LE-specific disorders include acute cutaneous LE, subacute cutaneous LE, and several forms of chronic cutaneous LE, including discoid LE. Skin biopsies are often helpful in differentiating LE-specific skin lesions from other disorders that can mimic them. Photoprotective measures and a number of drugs are useful in treating cutaneous LE.
Anti-Inflammatory Agents, Non-Steroidal↗
Systemic lupus erythematosus and lupus syndromes in Senegal. A retrospective study of 30 patients seen over 10 years.
OBJECTIVE: To define the presenting manifestations, course and prognosis of systemic lupus erythematosus in Senegal. PATIENTS AND METHODS: Thirty cases of systemic lupus erythematosus and lupus syndromes seen over a ten-year period were reviewed retrospectively. Nineteen patients met American College of Rheumatology criteria for systemic lupus erythematosus. All 30 patients were Senegalese-born black women. Mean age at diagnosis was 30 years (range, 16-73 years). RESULTS: Polyarthritis was the most common presenting picture (n = 8), followed by discoid lupus (n = 6). Eight per cent of patients had at least a combination of skin and joint symptoms at diagnosis. Prevalences of organ involvement were as follows: skin, 97%; joints, 97%; kidneys, 57%; serous membranes, 43%; nervous system, 23% and blood, 83%. Mean symptom duration at diagnosis was 24 months in systemic lupus erythematosus patients and 43 months in lupus syndrome patients. This significant diagnostic delay explains why as many as five of the 30 patients (17%) died either before or within five months of treatment initiation. Eight patients (32% of treated patients) who had a favorable course under therapy were lost to follow-up after discharge. The overall mortality rate was 26%. Renal failure was the main cause of death, followed by infectious and neurologic complications. CONCLUSION: We anticipate that the reported prevalence of lupus in Senegal will rise in the near future as a result of improvements in diagnostic tools and of increased interest for lupus among physicians. We also hope that improved detection of mild and early forms, together with close long-term follow-up of patients, will translate into a better overall prognosis.
Adolescent↗
Monozygotic twins concordant for idiopathic thrombocytopenic purpura and discordant for systemic lupus erythematosus and lupus nephritis.
We report on identical twin sisters with systemic lupus erythematosus and lupus nephritis after initial presentation as idiopathic thrombocytopenic purpura. The patients had diverse clinical signs, symptoms and pathological findings of lupus nephritis. The changes in antinuclear antibodies and anti-double-stranded DNA antibodies were quite different. We conclude that even with an identical genetic background and the same environment, the expression of systemic lupus erythematosus and subsequent progression to lupus nephritis in twins was distinct, and antinuclear antibodies and anti-double-stranded DNA antibodies are helpful for clinical monitoring.
Adolescent↗
[Chronic lupus erythematosus in lupus disease. Retrospective study of 136 patients].
OBJECTIVES: The aim of this retrospective study of 136 patients was to specify the natural history and the systemic prognosis of chronic cutaneous lupus erythematosus. It has been stated that in most of the cases, the disease only affects the skin. METHODS: From 10 October 1980 to 31 December 1990, 136 patients with the following criteria were included in this retrospective study: clinical signs suggestive of chronic cutaneous lupus erythematosus, characteristic histology, insufficient evidence for the diagnosis of systemic lupus erythematosus. RESULTS: The prevalence of systemic clinical involvement in the population under study was nearly the same as in the general population. The following biologic or immunologic abnormalities were quite common: leukopenia, lymphopenia, thrombopenia (significantly more frequent among patients having widespread chronic cutaneous lupus erythematosus), low titers of complement levels, positive antinuclear antibodies (usually at a low titer). Eleven out of the 136 patients developed systemic lupus erythematosus, most often over 5 years after the onset of the cutaneous lesions. Four cases out of these 11 had poor prognosis: renal and/or neurologic involvement. CONCLUSION: This data suggests that patients with chronic cutaneous lupus erythematosus could benefit from long-term follow up, since the course to systemic disease occurs in only a few. Usually, antimalarials used singly or in combination with topical steroids may lead to the clearing of the lesions. Thalidomide will occasionally be useful whenever the disease is unresponsive to the preceding measures.
Adult↗
Recurrent lupus nephritis in the second allograft of a patient with systemic lupus erythematosus.
Lupus glomerulonephritis is a common and serious complication of systemic lupus erythematosus (SLE) affecting up to 50% of lupus patients. Recurrent lupus nephritis is rare, complicating as low as 1% of the lupus transplant population according to some authors. However, it may be underreported with more realistic recurrent rates oscillating from 2.8 to 8.7%. We report the case of a patient with SLE who lost her first allograft 4 years after transplantation with a diagnosis of de novo fibrillary glomerulopathy. She underwent a second renal transplantation and her renal function was stable for the past 5 years. She now presented with skin rash, arthralgias and positive lupus serologies. Her creatinine was slightly elevated and proteinuria was also noted. A renal biopsy performed revealed a recurrent focal proliferative lupus nephritis (WHO III). Retrospectively, we believe that her first allograft was also lost to recurrent lupus nephritis. This is a unique case of recurrent lupus nephritis in the second allograft of a patient with SLE.
Adult↗
Lupus erythematosus.
Lupus erythematosus (LE) is a multisystem disease. Genetic predisposition, altered immunity, hormones, drugs, viruses, and ultraviolet light all may play a role in etiology. A wide range of cutaneous lesions occur, and variants such as subacute cutaneous LE, complement-deficient LE, and neonatal LE have recently been emphasized. Management of the LE patient, including appropriate diagnostic studies and therapy relevant to the dermatologist, is discussed in the review.
Antigen-Antibody Complex↗
Limiting dilution analysis of Epstein-Barr virus infectable B cells secreting anti-Ro/SSA and anti-La/SSB antibodies in neonatal lupus erythematosus and systemic lupus erythematosus.
Neonatal lupus erythematosus (NLE) is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies. In order to better determine the risk of delivery of a child with NLE, we examined the frequency of anti-Ro and anti-La antibody secreting cells in mothers of children with NLE and in mothers at risk to deliver a child with NLE. We established limiting dilution experiments, using EBV-infected peripheral blood mononuclear cells, from 10 mothers following delivery of a child with NLE and from six mothers with anti-Ro and anti-La antibodies who delivered an unaffected child. Supernatants were assessed, by Poisson analysis, to determine the frequency of IgG and IgM anti-Ro and anti-La antibody-secreting B cells. We found that the frequency of anti-Ro and anti-La IgM antibody secreting B cells was greater than the frequency of IgG antibody secreting B cells of the same autoantibody specificity. We found no correlation between serum IgM and IgG anti-Ro or anti-La antibody titres and their respective precursor cell frequencies. We found that the mothers of children with NLE who later developed SLE tended to have higher anti-Ro and anti-La antibody-committed B cells than did the mothers who remained well. Although anti-Ro and anti-La antibody precursor frequencies were similar within a patient, they varied significantly from patient to patient. We found that most of the experiments with a precursor frequency of < 1 per million were from mothers of children with NLE rather than the mother with SLE who delivered normal children. Overall, we found that, in anti-Ro and anti-La antibody-positive women, a low anti-Ro or anti-La antibody B cell precursor frequency tended to be associated with the birth of a child with NLE.
Antibodies, Antinuclear↗
Subacute cutaneous lupus erythematosus and discoid lupus erythematosus. Comparative histopathologic findings.
Subacute cutaneous lupus erythematosus (SCLE) is a recently described distinct subset of lupus erythematosus (LE) having characteristic clinical, serologic, and genetic findings. This study describes the histopathologic characteristics of SCLE and determines whether it could be differentiated from discoid lupus erythematosus (DLE) on histopathologic grounds alone. Biopsy specimens from 33 patients having either SCLE or DLE, as defined by strict clinical criteria, were examined without knowledge of the clinical diagnosis. Histologic discrimination between SCLE and DLE was accomplished in 82%. The specimens from DLE lesions had substantially more hyperkeratosis, basement membrane thickening, follicular plugging, and superficial and deep inflammatory cell infiltrate, while SCLE had more epidermal atrophy. The histopathologic differences between SCLE and DLE further support the concept that SCLE is distinct from DLE and should be considered a unique subset of LE.
Acute Disease↗
The dynamism of cutaneous lupus erythematosus: mild discoid lupus erythematosus evolving into SLE with SCLE and treatment-resistant lupus panniculitis.
We present a 47-year-old Caucasian female who initially presented with mild discoid lupus erythematosus that evolved into systemic lupus erythematosus with subacute cutaneous LE and treatment-recalcitrant lupus panniculitis. Conventional therapy with antimalarials, systemic steroids, azathioprine, cyclophosphamide, methotrexate, and pulse doses of methylprednisolone did not control the course of the disease. Cyclosporin-A treatment led to clinical improvement and maintained remission.
Antimalarials↗