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The specificity and clinical usefulness of the lupus band test.

Granular deposition of immunoglobulin at the dermoepidermal junction is characteristic, but not pathognomonic, of lupus erythematosus. When rigid criteria for the lumpus band test are used, a positive result is highly specific. A positive band test is found in clinically normal skin and lesional skin of systemic lupus patients, and in lesional skin of discoid lupus patients. A positive band test is found infrequently in clinically normal skin of patients with other connective tissue disorders. Cutaneous lupus may be difficult to separate from other morphologically similar disorders. The lupus band test is useful in differential diagnosis because other disorders have either a specific pattern of immunoglobulin deposition or no immunoglobulin deposition. The mechanisms of immunoglobulin deposition at the epidermal basement membrane zone are discussed as well as pertinent clinical correlations of the positive band test.

Antibodies, Antinuclear↗

Immunopathology of cutaneous human lupus erythematosus defined by murine monoclonal antibodies.

Skin biopsy specimens obtained from involved skin from sixteen patients with systemic and discoid lupus erythematosus were studied. Murine monoclonal antibodies with a biotin-avidin-horseradish peroxidase staining system were used. The findings consisted of a marked reduction in the number of epidermal Langerhans cells defined by surface antigens, reduced HLA-DR (Ia-like) antigens on the surface of dermal capillary endothelium, and mononuclear cell infiltrates characterized by a predominance of helper T lymphocytes and an increase in the number of mononuclear phagocytic cells. B lymphocytes were rarely identified. The number of T lymphocytes within the dermis correlated inversely with both the number of HLA-DR-positive epidermal Langerhans cells (p less than 0.01) and the HLA-DR staining of dermal capillary endothelium (p less than 0.01). These findings suggest that a T lymphocyte-mediated immune response associated with a reduction in Langerhans cells and capillary endothelium HLA-DR antigens is involved in the inflammatory process of lupus erythematosus skin.

Adult↗

How reliable is the basement membrane phenomenon in the diagnosis of systemic lupus erythematosus?

BACKGROUND: Although the basement membrane (BM) phenomenon is considered a good test to differentiate between systemic (SLE) and discoid lupus erythematosus (DLE), our observations question its reliability. METHODS: Direct immunofluorescence stain was done to detect immunoglobulins and complement deposits in 10 SLE patients and in 10 healthy controls. Specimens were taken from the normal skin of the dorsa of hands. RESULTS: The group of SLE patients showed deposits of IgG in 4, IgM in 7, IgA in 1, C3 in 7, and C4 in 1 patient. The group of healthy controls showed IgM in 2, C3 in 5, C4 in 2 cases, but no IgG or IgA deposits. CONCLUSION: A positive BM phenomenon test could be found in normal individuals and may be due to the effect of ultraviolet rays. We think that the BM phenomenon has a limited reliability in diagnosing SLE.

Basement Membrane↗

Circulating T- and B-cell abnormalities in cutaneous lupus erythematosus.

Seven patients with subacute cutaneous lupus erythematosus (SCLE), 11 patients with discoid lupus erythematosus (DLE), and 17 age-, sex-, and race-matched controls were examined for the number of circulating peripheral blood T and B cells, immunoglobulin (Ig) synthesizing cells, and Ig secreting cells. A significant alteration in T-cell subsets was detected only in SCLE patients who manifested a decreased number of OKT8 cells. Circulating B cells were significantly increased only in the SCLE patients. The percentage of Ig synthesizing and secreting cells was significantly increased in both the DLE and the SCLE groups. The somewhat unexpected increase in Ig synthesizing and secreting cells in the DLE patients could not be accounted for by antimalarial treatment or the concurrence of the HLA-DR3 phenotype. There was, however, a weak but significant correlation between the degree of B-cell activation in the DLE patients and the number of American Rheumatism Association criteria for the classification of systemic lupus erythematosus (SLE) possessed by these patients. These data demonstrate that abnormalities in B-cell function similar to those seen in SLE can also be found in a group of lupus erythematosus patients whose clinical disease expression is limited predominantly to the skin.

B-Lymphocytes↗

Anetoderma. Clinical findings, associations, and long-term follow-up evaluations.

In 16 patients with anetoderma, a clinicohistologic entity related to a local dermal defect of elastic tissue, old lesions did not heal, and new lesions often continued to form for many years, despite various forms of treatment. Systemic lupus erythematosus, which occurred in one patient, must be ruled out in patients with anetoderma. Discoid lupus erythematosus occurred later in one patient. Other associated findings, noted in one patient each, included cataract, congenital hip dislocation, congenital fusion of the vertebrae at C2-3, diverticulum of the midesophagus, Addison's disease (before the onset of anetoderma), and mitral valve prolapse. Our results and a review of the literature indicate that patients with anetoderma must be examined for associated eye, bone, heart, pulmonary, digestive tract, and endocrine abnormalities for a better assessment of their skin disorders.

Adolescent↗

[Aseptic bone necrosis in lupus disease].

Seven cases with aseptic necrosis in lupus disseminatus are presented being 9.70 per cent of 72 patients with confirmed systemic lupus, observed in the cours of 20 years. Six cases with lupus disseminatus are concerned--acute and subacute paroxysm a course and one case with chronic skin form--discoid lupus, complicated in the course of its evolution with aseptic necrosis of the right head of the femur. The cause for detection of the bone lesions in the cases presented, was the pain, especially manifested with the necrosis localized in the supporting bones of the lower limbs. The analysis of the state of the patients with systemic lupus reveals that all cases, are severe forms of the disease with multiorgan involvement, febrile reactions and the presence of vascular phenomena--Raynaud syndrome, coronary and cerebral arteriitis. High doses of corticosteroid preparations were used in the treatment of the disease due to its lasting progress (paroxysms of 8--12 months).

Adrenal Cortex Hormones↗

Congenital discoid lupus in the newborn.

A female infant, born to a 21-year-old mother with systemic lupus erythematosus, had cutaneous discoid lupus at birth. The lesions resolved spontaneously over the first few months and by the age of 1 year the infant's skin was normal. Other possible complications of this maternal disease are discussed and the need for caution in counselling mothers is recommended.

Female↗

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↗

Uses for immunofluorescence tests of skin and sera. Utilization of immunofluorescence in the diagnosis of bullous diseases, lupus erythematosus, and certain other dermatoses.

Sera and frozen biopsy specimens taken for immunofluorescence can be of diagnostic value in studies of vesiculobullous eruptions and connective tissue diseases. Appropriate handling of specimens is important. In cases of pemphigus and pemphigoid, findings of typical antibodies in sera and immunoglobulin deposits in skin sections serve to establish the diagnoses. Fluctuations in titers of pemphigus antibodies afford a prognistic guide. IgA deposits at the dermoepidermal junction in patients with vesiculobullous eruptions are diagnostic of dermatitis herpetiformis. Elevated titers of antinuclear antibodies, frequently with a peripheral pattern, and DNA antibodies as well as junctional deposits of immunoglobulins or complement or both in the apparently uninvolved skin are highly characteristic of systemic lupus erythematosus, more or less typical patterns of immunoglobulin and complement deposits in the lesion appear in discoid lupus erythematosus, porphyrias, and some other dermatoses.

Acantholysis↗

Anti-Ro/SSA antibodies. Association with a particulate (large speckledlike thread) immunofluorescent nuclear staining pattern.

Twenty-one patients with clinical and histopathologic evidence of subacute cutaneous lupus erythematosus and one patient with Sjögren's syndrome and vasculitis had anti-Ro/SSA (Sjögren's syndrome A) antibodies as demonstrated by double immunodiffusion assay using a saline extract of human spleen as the source of antigen. These serum samples were negative when tested for other nuclear antigens, including native DNA, Sm, and RNP. When tested for antinuclear antibodies (ANAs) by immunofluorescence, only ten of 22 serum samples were ANA positive on mouse liver substrate, while 18 of 22 had a positive ANA when HEp-2 tumor cells were utilized. With imprints of human spleen as test substrate, all 22 serum samples yielded a positive ANA result and a particulate (large speckledlike thread) staining pattern. Absorption of two of these serum samples with human spleen extract containing Ro/SSA antigen inhibited both the particulate staining pattern using human spleen imprints and the anti-Ro/SSA precipitin line by double immunodiffusion. These studies suggest that anti-Ro/SSA antibodies and antibodies producing the particulate nuclear staining pattern on human spleen imprints are either one and the same or closely paralleling antibody systems.

Absorption↗

Neonatal lupus erythematosus. A report of three cases associated with anti-Ro/SSA antibodies.

Three patients had neonatal lupus erythematosus syndrome. All three infants were girls who presented with characteristic skin lesions but without evidence of congenital heart block. All three mothers were asymptomatic at the time of delivery and have remained so until the present time. All mothers were positive for anti-Ro/SSA antibodies, as were the infants prior to 6 months of age. Anti-La/SSB antibodies were not present in either the mothers or the infants. HLA typing revealed that two of the mothers were positive for B8 and DR3, while the third mother was positive for B8 and DR2. Two of the infants were positive for B8 and DR3, while the third infant was positive for DR2 only.

Antibodies↗

Lupus erythematosus.

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Lupus Erythematosus, Discoid↗