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[Migrant eruptions in lupus erythematosus. Apropos of a case of lupus erythematosis gyratus repens].

Rare cases of migrant circinated eruptions occurring during the course of lupus erythematosus have been published so far. They have been classified in two categories: 1) supposed toxicodermias due to antimalarials; 2) real cases of erythema annulare centrifugum, mostly inaugurating the L. E. In an exceptional case, a migrating eruption looking like an erythema annulare with a complicated pattern on the trunk, neck and limbs, took place between two rashes of typical L.E. The exact nature of this eruption, clinically and histologically atypical, and its relation with the L.E. itself, have been discussed.

Aged↗

Antinuclear antibodies. II. The prognostic significance of nuclear immunofluorescent patterns in lupus erythematosus.

Nuclear immunofluorescent patterns in lupus erythematosus (LE) were correlated with the clinical picture. Patterns were classified into three groups of prognostic significance: (1) Poor-Shrunken peripheral fluorescence was found in patients with severe systemic lupus erythematosus (SLE), 60% demonstrating renal involvement. (2) Intermediate-This included the peripheral and therapy patterns, associated with 33% renal involvement in patients with SLE. (3) Good-Large and small speckle-like threads were seen in cases of very benign LE.

Antibodies, Antinuclear↗

Ethnic and geographical differences in systemic lupus erythematosus: an overview.

Systemic lupus erythematosus (SLE) is one of the most heterogeneous autoimmune disorders known. There is production of a variety of autoantibodies and patients present with a wide range of symptoms due to multiple organ involvement by the disease process. The underlying cause is not fully understood but it may involve genetic and environmental factors. It is interesting to note that while SLE is found worldwide, it is more commonly found in some countries, and within a country certain ethnic groups appear to be more susceptible to develop this condition than others. Additionally, the presentation and course of SLE appear highly variable between patients of different ethnic origins. For example, African-Americans and Orientals are believed to have a more severe disease than Caucasian whites. But are these ethnic and geographical differences real? If yes, they may provide investigators insight into the underlying pathoaetiology of this condition and pave the way to future research directions in lupus.

Anemia, Hemolytic, Autoimmune↗

Effect of glutathione S-transferase polymorphisms and proximity to hazardous waste sites on time to systemic lupus erythematosus diagnosis: results from the Roxbury lupus project.

OBJECTIVE: The high prevalence of systemic lupus erythematosus (SLE) among African American women may be due to environmental exposures, genetic factors, or a combination of factors. Our goal was to assess association of residential proximity to hazardous waste sites and genetic variation in 3 glutathione Stransferase (GST) genes (GSTM1, GSTT1, and GSTP1) with age at diagnosis of SLE. METHODS: Residential histories were obtained by interviewing 93 SLE patients from 3 predominantly African American neighborhoods in Boston. Residential addresses and locations of 416 hazardous waste sites in the study area were geocoded using ArcView software. Time-varying Cox models were used to study the effect of residential proximity to hazardous sites, GST genotype, and interaction between genotype and exposure in determining age at diagnosis. RESULTS: The prevalence of SLE among African American women in these neighborhoods was 3.56 SLE cases per 1,000. Homozygosity for GSTM1-null and GSTP1 Ile105Val in combination was associated with earlier SLE diagnosis (P = 0.03), but there was no association with proximity to 416 hazardous sites. Available data on specific site contaminants suggested that, at a subset of 67 sites, there was higher potential risk for exposure to volatile organic compounds (P < 0.05 with Bonferroni correction). GST genotypes had a significant interaction with proximity (P = 0.03) in analyses limited to these sites. CONCLUSION: There was no independent association between residential proximity to hazardous waste sites and the risk of earlier SLE diagnosis in this urban population. However, analysis of a limited number of sites indicated that the risk of earlier SLE associated with proximity to hazardous sites might be modulated by GST polymorphisms.

Adolescent↗

[Rare cutaneous manifestaions of lupus erythematosus. A clinical overview].

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations and has been the subject of many studies over several decades. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Consequently, this has led to the identification of subsets which have been defined by constellations of clinical and photobiological features, histological changes as well as laboratory abnormalities. Besides the characteristic classical forms such as systemic LE (SLE), subacute cutaneous LE (SCLE), and discoid LE (DLE), there are uncommon variants of LE which often lead to diagnostic difficulties. Bullous LE (BLE) and urticarial vasculitis are listed as characteristic but non-specific manifestations of systemic LE. LE tumidus (LET), LE hypertrophic/verrucous (LEHV), chilblain LE, and LE profundus (LEP) are uncommon subtypes of chronic cutaneous LE. Annular erythema and papulonodular mucinosis are further uncommon cutaneous manifestations of LE. This clinical review summarizes the typical features of the uncommon forms of LE in order to improve clinical diagnostic precision and to achieve a better differentiation of the subtypes.

Diagnosis, Differential↗

A case of severe neonatal lupus erythematosus without cardiac or cutaneous involvement.

Neonatal lupus erythematosus is a disease primarily characterized by cardiac and/or cutaneous involvement. Liver and haematological involvement occur in a few cases and these are normally considered to be mild and transient complications. The disease is suspected to be caused by transplacental passage of maternal autoantibodies, primarily anti-Ro/SSA and anti-La/SSB, from mothers with systemic lupus erythematosus. Here we report a child with neonatal lupus erythematosus who died from severe liver and haematological disease when 2 d old. The baby had no cardiac or cutaneous involvement. The mother has had two subsequent pregnancies. In the first she had a boy without certain signs of neonatal lupus erythematosus. In the next pregnancy the child developed congenital complete heart block and cutaneous lesions.

Antibodies, Antinuclear↗

Immunogenetic findings in cutaneous lupus erythematosus.

The serologic features and histocompatibility antigen associations of 93 patients with cutaneous lupus erythematosus are reported. Of them, 37 had chronic cutaneous lupus erythematosus and 56 had subacute cutaneous lupus erythematosus. Subacute cutaneous lupus erythematosus with an annular pattern occurred in 32 patients; 24 had a papulosquamous morphology. Sixty percent of patients with annular subacute cutaneous lupus erythematosus had anti-Ro antibodies. The incidence of this antibody in the other two groups was lower. In addition, patients with annular skin lesions had significant antigen association with HLA-B8 (37.5%; relative risk, 4.6) and HLA-DR3 (59.4%; relative risk, 4.3). There were no significant differences among chronic cutaneous lupus erythematosus and subacute cutaneous lupus erythematosus of the papulosquamous type and the control population for any HLA loci. Thus only patients with annular skin lesions represent a homogeneous subgroup in which there is a striking concordance of anti-Ro antibodies and the HLA-B8 and HLA-DR3 phenotypes.

Acute Disease↗

Subacute cutaneous lupus erythematosus associated with phenytoin.

Subacute cutaneous lupus erythematosus is a well recognized subset of systemic lupus erythematosus. It is characterized by a nonscarring, papulosquamous or annular eruption in a photosensitive distribution. Several cases, thought to be caused by drugs, have been reported. We report a case of subacute cutaneous lupus erythematosus caused by phenytoin, which has not previously been associated with this condition.

Aged↗

Immunogenetic study of three Japanese families with neonatal lupus erythematosus.

We encountered three Japanese families with neonatal lupus erythematosus. None of the three fathers showed any signs of collagen disease. The three mothers were found to suffer from Sjögren's syndrome; they all tested positive for anti-SSA and SSB antibodies and had lymphocyte infiltration into the small salivary gland. In two families, one child had neonatal lupus erythematosus while a sibling was normal; in the third family, both children had neonatal lupus erythematosus. Thus, a mother with positive anti-SSA and SSB antibodies can give birth to one infant with and one infant without or have two infants with neonatal lupus erythematosus. We conducted HLA typing of all 12 members of the three families in order to clarify the immunologic factors involved. We found no increased frequency of any HLA phenotype in the three mothers and their four children with neonatal lupus erythematosus; however, HLA-DR4 was present in three of the children with neonatal lupus erythematosus.

Adolescent↗

Cytological aspects of pleural, peritoneal and pericardial fluids from patients with systemic lupus erythematosus.

Serous effusions of nine of 33 patients with systemic lupus erythematosus contained lupus erythematosus (LE) cells, identifiable in Papanicolaou-stained smears, wet films stained with toluidine blue, and cell blocks stained with haematoxylin and eosin. Specimens in which LE cells were found contained at least a moderate number of polymorphonuclear neutrophilic leucocytes. Most specimens containing LE cells also contained cells that resembled LE cells (tart cells), which appeared to be small macrophages that had phagocytosed a non-homogenized nucleus of a cell that had undergone degeneration. In 34 years of cytologic practice we have recognized LE cells in serous effusions only from patients who were already diagnosed as having systemic lupus erythematosus.

Adolescent↗

Perturbation of experimental ultraviolet light-induced erythema by passive transfer of serum from subacute cutaneous lupus erythematosus patients.

Several lines of investigation have implicated anti-Ro/SS-A antibody in the pathogenesis of photosensitive forms of cutaneous lupus erythematosus such as neonatal lupus erythematosus and subacute cutaneous lupus erythematosus. To further explore this possibility, we have developed a quantitative, experimental system for examining the effect of passively transferring anti-Ro/SS-A antibody-containing and antibody-deficient subacute cutaneous lupus erythematosus patient sera on one aspect of cutaneous photoreactivity, UV-induced erythema. Laser-Doppler velocimetry was used to quantitate the microvascular flow rates in normal control, disease control (rheumatoid arthritis, discoid lupus erythematosus), and subacute cutaneous lupus erythematosus serum-injected guinea pig skin test sites before and after combined ultraviolet B and A radiation from a solar simulator. Results, expressed as change in milli-electron voltage (perturbed milli-electron volts after irradiation minus baseline milli-electron volts before irradiation), revealed that subacute cutaneous lupus erythematosus serum injections consistently resulted in greater UV-induced microvascular flow rates than those elicited by normal or disease control serum injections. Anti-Ro/SS-A containing subacute cutaneous lupus erythematosus sera produced the greatest flow rates observed in this study. Earlier studies have suggested that the pathogenesis of lupus photosensitivity is very likely multifactorial. Our current data suggest that anti-Ro/SS-A autoantibody or other closely related humoral elements should also be considered among the factors which might contribute to this clinical phenomenon.

Acute Disease↗

Successful treatment of hypertrophic lupus erythematosus with isotretinoin.

A patient with systemic lupus erythematosus had the additional finding of hypertrophic lupus erythematosus. The lesions cleared with an 11-week course of isotretinoin alone. She has remained without recurrence for 9 months. This is the first reported case of total resolution of hypertrophic lupus erythematosus with a short course of isotretinoin.

Adult↗

[Discoid lupus erythematosus involving the eyelids: 4 cases].

BACKGROUND: Discoid lupus erythematosus is a chronic skin disease frequently involving the face, scalp and ears. Palpebral lesions are rare. We report 4 cases of this uncommon localization. In one case, palpebral lesions were the sole manifestation of the discoid lupus erythematosus. CASE REPORTS: Four patients aged between 26 and 45 Years had lesions of the lower eyelid as erythematous, infiltrated and scaly plaques. One patient, without diagnosis despite 2 skin biopsies of lesion, presented with a 2-Year history of blepharitis in the absence of any other cutaneous abnormality. In one case, chronic blepharitis has been present for 4 Years and associated with inflammatory arthralgia. Alopecia occurred 4 Years after the onset of blepharitis and led to the diagnosis of discoid lupus erythematosus. In 2 cases, blepharitis was associated with typical cutaneous lesions of discoid lupus erythematosus. Antimalaria drugs were very effective in 3 cases. In one patient the antimalaria drug failed, but thalidomide was effective. DISCUSSION: The location of lesions on the eyelids in the absence of any other cutaneous abnormality is rare and can easily lead to the misdiagnosis of discoid lupus erythematosus. Most Authors mention a predilection of the lesions to the inferior portion of the eyelid, more specifically to the external third. The involvement of the eyelids with permanent scarring and severe eye impairment is explained by the long duration of the disease without diagnostic and appropriate treatment. Clinical examination is highly consistent with discoid lupus erythematosus: the lesions typically present as well-circumscribed, erythematous plaques with telangiectasia and scales and atrophy in long-standing disease. Antimalaria drugs are remarkably effective.

Adult↗

Update on vascular disease in systemic lupus erythematosus.

PURPOSE OF REVIEW: Young women with systemic lupus erythematosus have strikingly high rates of coronary heart disease. Current knowledge indicates that atherosclerosis is an active inflammatory and immune-mediated process. Therefore, the chronic inflammation and immune dysregulation characteristic of systemic lupus erythematosus undoubtedly contribute to the accelerated vascular disease seen in these patients. Carefully considering what is known about atherogenesis in the general population will provide clues to unraveling the complexity of why systemic lupus erythematosus and atherosclerosis are linked so frequently. RECENT FINDINGS: Inflammation is involved in all aspects of atherogenesis from the initial endothelial "response to injury," to foam cell formation leading to the atherosclerotic lesion, to the rupture of the "vulnerable" fibrous cap, resulting in the acute coronary syndrome and potentially in death. The authors review how factors commonly seen in systemic lupus erythematosus or inherent to the underlying disease mechanism may contribute to each of the stages of atherogenesis. SUMMARY: Our focus on the causes of vascular disease in systemic lupus erythematosus must now include nontraditional risk factors such as immune and inflammatory mediators. With the advent of noninvasive screening tools for atherosclerosis, we are better equipped to measure subclinical vascular disease and associated risk factors, including immune and inflammatory mediators. When considering strategies for preventing premature cardiovascular disease in systemic lupus erythematosus, modifying immune and inflammatory risk factors will likely become a major component of the program in addition to modifying the current traditional risk factors.

Adult↗

Activation/inactivation of the classical pathway of complement in non-lesional skin of patients with systemic lupus erythematosus.

BACKGROUND: The link between the lupus band and pathogenesis remains controversial, because immunoglobulins and complement components, including the membrane attack complex, can be found in both lesional and non-lesional skin of patients with systemic lupus erythematosus (SLE). The expression of proteins that regulate complement has not been previously investigated in the skin of patients with SLE. AIM: The aim of this study is to compare the expression of protectin (CD59), which demonstrates the activation of the classical pathway of complement, in non-lesional skin obtained from patients with SLE with its expression in normal skin. This may help us explain the link between the lupus band and pathogenesis of cutaneous lupus erythematosus. METHODS: An indirect immunofluorescence technique was performed in order to provide unequivocal evidence for the activation of complement via the classical pathway and to compare the expression of CD59 in non-lesional skin from patients with SLE with normal skin samples obtained from healthy people. RESULTS: The activation of the classical pathway of complement was demonstrated in non-lesional skin in more than 90% of SLE patients investigated in this study. Staining intensity of the complement regulatory protein CD59 was markedly increased in the majority of non-lesional skin samples obtained from patients with SLE, compared to that from normals. CONCLUSIONS: CD59 is overexpressed in non-lesional skin in which complement activation has occurred. It seems likely that an increased and continuous CD59 expression may be important for maintaining the integrity of the skin BMZ during inflammatory responses involving complement activation in SLE skin. Alahlafi A, Wordsworth P, Wojnarowska F. Activation/inactivation of the classical pathway of complement in non-lesional skin of patients with systemic lupus erythematosus.

CD59 Antigens↗

Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.

Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). We report here 9 patients with chilblain cutaneous lesions, 6 of them were affected by SLE and 2 by SCLE. The onset of CL preceded the diagnosis of LE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the remaining 4 cases. Raynaud's phenomenon and photosensitivity were other prominent clinical features in patients with CL. Nailfold capillaroscopy revealed pathological changes in every patient examined. ANA and anti-SSA/Ro antibodies were detected in all nine patients. Anti-SSB/La were detected in 2 cases, anti-Sm in one case, and anti-Sm and anti-RNP in a one case. Antibodies to dsDNA and complement consumption were found in the six patients with SLE. The fine specificity of anti-SSA/Ro was determined by immunoblotting: anti-60kD and anti-52 kD were detected in three sera, anti-60kD alone in 5 sera, while one serum did not blot. In conclusion, the present study suggests that chilblain LE is associated with SSA/Ro autoantibodies, as is SCLE, hypergammaglobulinemic purpura and neonatal lupus erythematosus.

Adolescent↗

Interactions of prednisolone and other immunosuppressants used in dual treatment of systemic lupus erythematosus in lymphocyte proliferation assays.

Systemic lupus erythematosus is an autoimmune disease primarily affecting women. Currently, systemic lupus erythematosus therapy is suboptimal due to adverse effects of immunosuppressants, particularly corticosteroids. This study determines the single effects of prednisolone, dehydroepiandrosterone, bromocriptine, tamoxifen, mycophenolic acid, 2-chloro-2'-deoxyadenosine, azathioprine, and chloroquine on lectin-stimulated proliferation of human T lymphocytes, as well as determining whether there are interactions in the joint effects of prednisolone and these agents. The T lymphocytes from the whole blood of 10 middle-aged women were stimulated by phytohemagglutinin and cultured with varying drug concentrations. The Hill function was used to evaluate single-drug response data. Isobolograms were constructed to qualitatively analyze interactions. Parametric analysis based on competitive and noncompetitive interaction models was further applied to quantify the joint interactions and predict steroid-sparing potential. The surface interaction parameter (psi) estimated from parametric analysis was in concordance with isobolographic inspection for all interactions studied. All interactions favored the noncompetitive model. Results suggest that dehydroepiandrosterone is additive in its effect with prednisolone, whereas tamoxifen interacts synergistically, both providing steroid-sparing effects. Novel immuno-suppressants such as mycophenolic acid may still provide added pharmacologic benefit during therapy despite a slight antagonistic interaction with prednisolone. These studies help rationalize actual or potential use of other drugs with prednisolone in the treatment of systemic lupus erythematosus.

Adult↗