The relationship between discoid lupus erythematosus and systemic lupus erythematosus. A hypothesis.
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Cases of a newborn infant with an eruption clinically and histologically consistent with lupus erythematosus and of his mother developing acute disseminate lupus erythematosus 11 months after delivery are presented. It is urged that in the future in cases of death of a fetus of a mother who has lupus erythematosus of any type or who gives a history of this disease, the fetus be examined for stigmata of lupus erythematosus. The possibility of a transmittable etiological agent of lupus erythematosus from mother to fetus is suggested.
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A patient with chronic discoid lupus erythematosus was admitted with fever, arthralia, pleuropericarditis, and a history of leukopenia. He was initially believed to have systemic lupus erythematosus (SLE), but extensive evaluation showed negative immunologic studies and the presence of acid-fast organisms on pericardial biopsy specimens with cultures positive for Mycobacterium tuberculosis. Discoid lupus erythematosus patients with extracutaneous manifestations should be carefully studied for concurrent illness, especially when serologic evidence fo SLE is negative.
Discoid lupus erythematosus (DLE) is a chronic skin disease that may affect the eyelids. Unless suspected, these lid lesions may resemble chronic blepharitis and persist for years. We present the clinicopathologic features of DLE from the lids of seven patients, electron microscopic features of one case, and a review of 31 previously reported cases.
Discoid lupus erythematosus (DLE) is rare in childhood. We report the case of a 15-month-old female infant who presented with erythematous telangectatic lesions and photosensitivity involving the sun-exposed areas. Histological examination confirmed the diagnosis of DLE. Direct immunofluorescence (DIF) on lesional skin showed granular IgM deposits along basement membrane zone. Laboratory tests were normal. External photo-protection and topical corticosteroids lead to complete healing. Summer recurrences that responded to topical corticosteroids were noted but there was no progression to systemic lupus erythematosus. Several authors note the absence of female predominance in children with DLE; prevalence of photosensitivity is controversial. Histological confirmation of DLE is easy and important for diagnosis. DIF is not specific but can be helpful in establishing the diagnosis of DLE. Laboratory tests rarely show low titers of antinuclear antibodies. Treatment is based on sun avoidance and photoprotection. Topical corticosteroids are indicated for active lesions. For resistant cases antimalarials are the treatment of choice. Progression to SLE is probably more frequent in children than in adults.
A patient with discoid lupus erythematosus has developed porphyria cutanea tarda. The combination of these diseases may be explained by similar changes in the free-radical processes in the body, induced by sunlight.
The pathophysiology of discoid lupus erythematosus is presented, along with a case of nasal reconstruction using a variety of techniques.
Discoid lupus erythematosus (DLE) is a rare disorder in childhood, with 22 cases reported in the English-language literature. Less than 2% of patients with DLE have an onset before 10 years of age. We describe two children with DLE and lupus profundus with an onset of the disease at the ages of 11 and 15 years and focus on likely histopathologic differences between DLE in children and in adults. Histopathologic characteristics for childhood DLE might be an intense periadnexal and perivascular infiltrate extending into the interstitium and subcutaneous tissue consisting of lymphocytes, histiocytes, eosinophilic granulocytes, and plasma cells and lacking epidermal atrophy. The diagnosis of DLE in our two patients was established by clinicopathologic correlation based on clinical presentation, histologic and immunofluorescent findings in skin biopsy specimens, and the absence of clinical and laboratory evidence of systemic involvement. Therapy with antimalarials resulted in reduction of the skin lesions, but in one patient severe lipoatrophy occurred.
Ten patients with classic discoid lupus erythematosus of the face associated with verrucous, papulonodular lesions on the arms and hands were studied by electron microscopy. The ultrastructural findings on the verrucous lesions included apoptotic keratinocytes, intraepidermal lymphocytes, and gapping, detachment, and reduplication of the basal lamina. Also, tubuloreticular inclusion bodies were present in the endothelial cells. These observations, together with the clinical, histopathologic, and immunofluorescence findings, suggest that the verrucous lesions represent a rare, but distinct, variant of chronic discoid lupus erythematosus.
Seventy-one patients with discoid lupus erythematosus were studied for anatomic regional variations in the lupus band test. The test was positive in 82% of biopsies from the scalp, face, neck, and upper extremity. Only three out of fourteen biopsies (21%) from the trunk were positive. Discoid lupus erythematosus could be confirmed or strongly suspected in the light microscopic sections from all cases. Truncal lesions should not be selected for the lupus band test in discoid lupus erythematosus if there are other choices.
Although the pathology of discoid lupus erythematosus is well documented the causative agents are not known. Here, we report the identity of the target antigen of an autoantibody present in high titre in the serum of a patient with discoid lupus erythematosus. We have demonstrated that the antigen is enolase; first, because it has properties consistent with this glycolytic enzyme (47,000 MW, cytosolic localization and ubiquitous tissue distribution). Secondly, limited amino acid sequence determination after trypsin digestion shows identity with alpha-enolase. Finally, the autoimmune serum immunoblots rabbit and yeast enolase and predominantly one isoelectric form of enolase (PI approximately 6.1). These results indicate that the reactive autoepitopes are highly conserved from man to yeast. The results also suggest that the autoantibodies are most reactive to the alpha-isoform of enolase, although it is possible that they may also be reactive with gamma-enolase, and have least reactivity to beta-enolase. The anti-enolase autoantibodies belong to the immunoglobulin G1 (IgG1) isotype. This is the first report of IgG1 autoantibodies to evolutionarily conserved autoepitopes of enolase in the serum of a patient with discoid lupus erythematosus. Previous reports of autoantibodies to enolase have suggested associations with autoimmune polyglandular syndrome type I and cancer-associated retinopathy. This report and an earlier report of what is likely to be enolase autoantibodies in two patients without systemic disease suggest that enolase autoantibodies have a broad association and are not restricted to any particular disease.
BACKGROUND: Discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) are chronic inflammatory diseases of unknown aetiology; the relationship of DLE with SLE has been a subject of debate for many years. OBJECTIVES; To find evidence for systemic immune activation in DLE by analysis of the immunophenotypic profiles of circulating lymphocytes, and to compare these changes with those in patients with SLE. METHODS: The immunophenotypic profile of peripheral blood lymphocyte subsets from 23 DLE patients without clinical or laboratory evidence of systemic disease, 25 SLE patients and 38 healthy donors was characterized by two-colour immunofluorescence flow cytometry analysis. None of the patients was receiving corticosteroid or immunosuppressive treatment. RESULTS: Patients with DLE had increased numbers of circulating HLA-DR+ CD3+ T cells and HLA-DR+ CD4+ T cells, indicating systemic T-cell activation, and an expansion of CD5+ CD19+ B cells. Decreased numbers of T-cell subsets expressing the differentiation markers CD11b and CD16/56, and of CD16/56+ natural killer cells were also found. In SLE, the changes were similar but more pronounced. In addition, a profound CD4+ T-cell lymphopenia and an increase of HLA-DR+ CD8+ T cells were found only in SLE. CONCLUSIONS: Our data provide evidence for systemic activation of the cellular immune system in patients with purely cutaneous DLE. Similarities in the lymphocyte immunophenotypic profiles in patients with DLE compared with SLE suggest that there are common immunopathological processes in these two conditions.
PURPOSE: Discoid lupus erythematosus (DLE) is an autoimmune disorder that usually affects the sun-exposed skin. Periocular involvement occurs uncommonly and may progress from eyelid erythema to scarring and madarosis. METHODS: Observational case report. RESULT: A case of DLE that presented with madarosis alone in the absence of preceding skin erythema and scarring. CONCLUSION: Our case demonstrates that DLE may present with madarosis alone in the absence of a history of preceding erythema and scarring. Discoid lupus erythematosus should therefore be considered as a differential diagnosis in chronic blepharitis that persists despite usual medical management and eyelid hygiene. Biopsy should be considered in the presence of clinical features such as erythematous scale on the face and alopecia and sent for direct immunofluorescence staining.
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