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PubMed2004

Lupus erythematosus tumidus.

BACKGROUND: Lupus erythematosus tumidus (LET) is a rare form of chronic cutaneous lupus erythematosus that characteristically presents as a succulent, erythematous plaques on sun-exposed areas. The histopathological change that primarily distinguishes LET from other variants of cutaneous lupus erythematosus is the lack of alterations of the dermo-epidermal junction and epidermis. Our purpose was to describe 15 cases of LET from Thailand. METHODS: The records of 15 patients diagnosed with LET at Srinagarind Hospital Medical School between 1995 and 2002 were reviewed on the following aspects: clinical manifestation, laboratory investigation, direct immunofluorescence study, phototesting, histopathological findings, clinical course, and treatment. RESULTS: All 15 patients consisted of 10 females and five males. The mean age at onset was 38.8 years. The lesions more commonly affect the face and trunk. One patient had concomitant discoid lupus erythematosus and two developed systemic involvement 4 and 6 months after LET. Direct immunofluorescence studies all showed negative results. Positive phototest reactions were detected in five of 10 tested patients: four with ultraviolet A and B and one with ultraviolet A only. Most patients responded well to chloroquine phosphate and the remainder successfully improved with methotrexate, topical and systemic corticosteroids. CONCLUSION: Our data demonstrate the features of LET that are repeatable and characteristic. Lupus erythematosus tumidus in this study was more common in women, and photosensitivity reactions of the patients were seen less frequently compared with those in the earlier report of Kuhn et al. Furthermore, two of 15 patients exhibited systemic involvement in the course of their disease.

Adolescent↗
PubMed1975

Discoid lupus erythematosus as part of a larger disease spectrum. Correlation of clinical features with laboratory findings in lupus erythematosus.

This study compares the immunologic features of a homogeneous group of patients with discoid lupus erythematosus (DLE) strictly limited to the skin (group 1) with those of patients with active discoid skin lesions plus visceral involvement (group 2) and with those of lupus erythematosus (LE) patients with proliferative glomerulonephritis (group 3). Positive antinuclear antibody (ANA) was found in 4% of group 1, 93% of group 2, and 100% of group 3. Low total hemolytic complement (CH50) was found in 4% of group 1, 47% of group 2, and 100% of group 3. Antibodies to native DNA (nDNA) were not found in group 1, were rarely found in group 2, and were present in nearly all patients in group 3. No group 1 patient had subepidermal immunoglobulin deposits in normal skin, 20% of group 2 had this finding, and 100% of group 3 had this finding. The ability to develop chronic discoid skin lesions appears to be associated with a reduced incidence of immunologic parameters of disease activity. The data suggest that patients with active discoid skin lesions rarely have severe renal disease.

Antibodies, Antinuclear↗
PubMed2004

Lupus erythematosus induced by medications, ultraviolet radiation, and other exogenous agents: a review, with special focus on the development of subacute cutaneous lupus erythematosus in a genetically predisposed individual.

Exogenous agents implicated in or suspected of precipitating subacute cutaneous lupus erythematosus (SCLE) and lupus erythematosus (LE) are reviewed. An illustrative case of environmentally induced SCLE is presented. A previously healthy 30-year male homozygous for the tumor necrosis factor-alpha (TNF-alpha) 308. A promoter allele developed SCLE after spending several hours removing fertilizer- and pesticide-containing hay from an agricultural barn in the springtime. The cutaneous eruption soon resolved, only to reappear 3 weeks later on the day the patient re-entered the barn. An environmental agent present in the barn, coupled with springtime ultraviolet light, likely triggered the disease in this immunogenetically susceptible individual.

Adult↗
PubMedDate not supplied

[Oral lesions in systemic lupus erythematosus. II. The clinical and therapeutic aspects of lupus erythematosus].

The authors describe the most common clinical characteristics and main parameters of SLE. They describe the currently most therapeutic protocol with particular reference to circulating immune complex study on patients with SLE, and they examine different prospects of applicability and of prognostic result. The aim of this paper is to provide an outline of the clinical diagnosis and the immunobiological significance of the nucleoprotein autoantibodies and of the Ro/SSA antigen-antibody system and the relationship between circulating anti Ro/SSa antibody levels and skin disease activity in SLE.

Adrenal Cortex Hormones↗
PubMed1995

Neonatal lupus erythematosus: discordant disease expression of U1RNP-positive antibodies in fraternal twins--is this a subset of neonatal lupus erythematosus or a new distinct syndrome?

Neonatal lupus erythematosus (NLE) is an uncommon disease that is manifested by cutaneous lesions, cardiac conduction defects, or both, that appear in utero or shortly after birth. In approximately 95% of patients, anti-Ro antibody (Ro[SS-A]) has been identified and has become the serologic marker for NLE. Since 1987 there have been four reported cases of Ro- and anti-La antibody (La[SS-B])-negative, U1RNP antibody-positive, NLE. Our affected twin, as well as all other infants with U1RNP-positive NLE, had cutaneous lesions similar to those in Ro-positive NLE, although they lacked systemic abnormalities, including cardiac conduction defects. HLA typing of mothers with infants with U1RNP-positive NLE revealed the presence of HLA-DR4, DQw1, or DQw3 phenotypes. Our typing confirms these findings. As with Ro-positive NLE, no distinct HLA associations were demonstrated in the infants. Unlike Ro-positive mothers, all mothers with a U1RNP-positive infant with NLE had connective tissue disease at the time of the diagnosis and had a different spectrum of disease. We describe the clinical, serologic, and immunogenetic findings in the first reported case of U1RNP-positive NLE in dizygotic twins in whom the NLE disease expression was discordant.

Adult↗
PubMed1988

Successful pregnancy in systemic lupus erythematosus with an untreated lupus anticoagulant.

A lupus anticoagulant in pregnancy has almost always been associated with an adverse outcome. In previous reports of successful pregnancy in patients with a lupus anticoagulant, whether treated or untreated, all were complicated by premature delivery, and many were complicated by preeclampsia or placental insufficiency. Four patients with systemic lupus erythematosus and an untreated lupus anticoagulant, had an uncomplicated pregnancy that resulted in a live birth at term. The circulating anticoagulant persisted throughout the pregnancy in three patients, and disappeared spontaneously during pregnancy in the fourth patient. As pregnancy outcome is unpredictable, the best treatment of these patients remains to be determined by controlled studies.

Adult↗
PubMed1983

Lupus erythematosus cell preparation, antinuclear factor and antideoxyribonucleic acid antibody incongruity in systemic lupus erythematosus.

'Total antinuclear antibody' (ANF) is detected by the fluorescent antinuclear antibody technique which is a screening test, positive in 99% of systemic lupus erythematosus (SLE) sera. The LE factor (positive in 75% of SLE sera), like the anti-DNA antibody, is an antinuclear antibody but directed against DNA-histone. ANF-negative SLE is a clinical entity with absence of these antibodies. A false negative ANF, in the presence of high titre anti-DNA antibody and/or LE cells, is illustrated in two cases of SLE. Postulated mechanisms for this phenomenon are interference in ANF detection by rheumatoid factor, and the prozone effect on the immunofluorescent tests.

Adult↗
PubMedDate not supplied

Linear childhood discoid lupus erythematosus following the lines of Blaschko: a case report with review of the linear manifestations of lupus erythematosus.

Seventeen cases of childhood discoid lupus erythematosus (DLE) have been previously reported in the literature. We describe the first reported case of childhood linear DLE following the lines of Blaschko. The clinical and histologic characteristics of childhood DLE are discussed and a review of the linear manifestations of childhood LE is presented.

Biopsy↗
PubMed1992

[Antibodies to poly (adenosine diphosphate-ribose) in systemic lupus erythematosus and drug induced lupus].

The difference between anti poly (ADP-ribose) antibodies was studied in patients with systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS) and drug-induced lupus (DIL). Radioimmunoassay showed that high concentrations of anti poly (ADP-ribose) antibodies (10.3-22.2%) were induced by phenobarbital, phenytoin, valproic acid (anti-epileptic agent) and procainamide (anti-arrhythmic agent). Poly (ADP-ribose) antibodies were separated by hydroxylapatite column chromatography. The average chain length of the polymer consisted of 2.4, 10.8 and 28.2 ADP-ribose units. Anti poly (ADP-ribose) antibodies from patients with SLE and PSS reacted with 2.4, 10.8, and 28.2 ADP-ribose units in RIA, but those from patients with DIL reacted only with 28.2 ADP-ribose units in RIA. The binding specificity of anti poly (ADP-ribose) antibodies from pregnant women was found to be very similar to that of the antibodies from case of DIL. The present results clearly demonstrated that anti poly (ADP-ribose) antibodies found in systemic autoimmune diseases bound not only to poly (ADP-ribose) with an average chain length of more than 20 ADP-ribose units, but also to oligo (ADP-ribose) with an average chain length of about 2 ADP-ribose units. Anti poly (ADP-ribose) antibodies found in DIL cases and pregnant women, however, bound only to poly (ADP-ribose) with an average chain length of more than 20 ADP-ribose units.

Antibodies↗
PubMed1999

[A case report of systemic lupus erythematosus patient with severe lupus retinopathy and antiphospholipid antibody].

An eleven-year-old boy with systemic lupus erythematosus (SLE) developed severe bilateral lupus retinopathy when he was in active stage of SLE. The patient, who had suffered from SLE for 3 years, was admitted to our hospital because of high grade fever, systemic lymphadenopathy, leukopenia, elevation of erythrocyte sedimentation rate and hypocomplementemia. The dose of prednisolone was increased considering he was exacerbated of SLE, however, the convulsion as CNS lupus occurred to him. After the event he noted loss of vision in his bilateral eyes. The ophthalmologic examination revealed the lesions of cotton-wool spots, retinal vessel dilatations and diffuse occlusions of the retinal arterioles and venules which were compatible with lupus retinopathy. Although the coagulation time was normal, antiphospholipid antibodies were positive at the time of ocular involvement. Careful attention should be paid to the occurrence of lupus retinopathy when the patients with SLE developed in the active stage or CNS lupus, especially they have antiphospholipid antibodies.

Antibodies, Antiphospholipid↗