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Systemic lupus erythematosus presenting as panniculitis (lupus profundus).

Six patients are described in whom panniculitis was a major manifestation of systemic lupus erythematosus. These patients were seen in a combined-clinics population of 270 patients with systemic lupus erythematosus for an incidence of approximately 2%. Panniculitis was the first symptom of systemic lupus erythematosus in three of these patients indicating that systemic lupus erythematosus should be considered as an underlying cause in patients with panniculitis or Weber-Christian's disease. Analysis of these six cases and those previously reported suggests that the addition of hydroxychloroquine to the treatment regimen may be beneficial in lupus panniculitis.

Adolescent↗

An appraisal of tests for native DNA antibodies in connective tissue diseases. Clinical usefulness of Crithidia luciliae assay.

Antibody to native DNA was measured by five techniques: the Crithidia luciliae immunofluorescence (CL-IF) test; filter radioimmunoassays using either untreated human KB DNA, endonuclease-treated KB DNA, or a synthetic polynucleotide (poly dAT); and the Farr immunoprecipitation assay using KB DNA. The specificity and sensitivity of the CL-IF assay was similar to that of the filter radioimmunoassay procedures using KB DNA. The CL-IF test showed an increased frequency of positive tests in patients with active disease and severe renal involvement. In patients with severe renal involvement, high titers of serum nDNA antibodies were measured by this procedure. A unique advantage of the CL-IF test was its ability to identify complement-fixing nDNA antibody. The presence of such antibody was correlated with high antibody titer and the presence of severe renal disease. The CL-IF assay is a simple and useful procedure for measurement of anti-nDNA.

Antibodies, Antinuclear↗

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↗

Immunohistopathology of light-induced skin lesions in lupus erythematosus.

Recently we have been able to induce pathological skin reactions with UVB, UVA and visible light in patients with lupus erythematosus (LE). The pathological skin reactions had the appearance of spontaneously developed LE lesions. In the present study, using patients with polymorphic light eruption as controls, we subsequently investigated what types of immunohistochemical abnormalities were found in these lesions. It was shown that in the induced skin lesions, phenotypically similar inflammatory cells were found as in spontaneously evolved lesions. Granular deposits of immunoreactants, as found in most spontaneously evolved LE lesions, occurred in 12 out of 16 LE patients 7-10 days after onset of the artificial irradiation. The dermal infiltrates in light-induced LE lesions differed mainly from those in polymorphic light eruption, by the amounts of CD1+ cells (Langerhans' cells). In polymorphic light eruption, the relatively large amount of these cells suggests an active migration of antigen-presenting cells, a mechanism apparently not operative in LE. Our results underline the importance of the pathogenic action of light in LE.

Antibodies, Monoclonal↗

Complete heart block as a rare complication of treatment with chloroquine.

Antimalarials are well established disease modifying antirheumatic drugs. A rare and underappreciated treatment difficulty is cardiac complication, in particular conduction disturbances. We describe 2 more patients that developed complete heart block after high dose, longterm treatment. Patient 1, a 73-year-old woman with longstanding rheumatoid arthritis, had taken chloroquine (250 mg/day) for 12 years when she developed complete heart block requiring a permanent pacemaker. Patient 2, a 40-year-old woman with discoid lupus erythematosus, was taking chloroquine from 1979 until 1996. Depending on the clinical disease activity, she intermittently increased the dose from 250 to 750 mg/day. In 1994, she developed complete heart block and a permanent pacemaker had to be implanted. Intensive investigations in both cases did not reveal another underlying cause for conduction disturbances; the atrioventricular block was probably due in both cases to chloroquine related cardiac toxicity. This toxicity seems to be restricted to longterm, high dose treatment; however, it should be kept in mind in patients with preexisting conduction disturbances during longterm treatment.

Adult↗

The utility of the lupus band test on sun-protected non-lesional skin for the diagnosis of systemic lupus erythematosus.

OBJECTIVE: The sensitivity and specificity of the lupus band test was evaluated, using three different criteria, on sun-protected non-lesional skin for the diagnosis of systemic lupus erythematosus (SLE). In addition, the sensitivity and specificity of the lupus band test was compared with those of other laboratory tests used in the diagnosis of SLE. METHODS: Sun-protected non-lesional skin biopsies from 65 patients (F 50; M 15; mean age 41 yrs.) with specific cutaneous manifestations of lupus erythematosus (LE) and from 18 patients with other dermatologic diseases (F 11, M 7; mean age 40 yrs.) were tested using the direct immunofluorescent technique. RESULTS: The sensitivity and specificity of the lupus band test was 10.5% and 97.8% respectively using the strict criterion of the presence of two different immunoreactants. The sensitivity and specificity were 52.6% and 69.5% respectively based on the presence of two different immunoreactants and were 78.9% and 47.8% based on the presence of only one immunoreactant. The highest sensitivity was found for ANA (100%). The specificity of all the laboratory abnormalities was particularly high, varying from 82.8% to 100%, except for ANA antibodies which showed a specificity of 65.2%. CONCLUSIONS: A positive lupus band test on sun-protected non-lesional skin (even if showing the presence of only one immunoreactant at the dermo-epidermal junction) represents a useful and specific criterion for identifying patients with LE. However, this test is not useful in distinguishing between cutaneous lupus patients with systemic involvement and those without systemic involvement.

Adult↗

[T- and B-lymphocytes in the so-called collagenoses].

Lymphocytes of peripheral blood of patients with systemic lupus erythematosus, scleroderma and dermatomyositis were investigated, and relatively, with regard to T- or B-cell characteristics. T-lymphocytes of most patients with SLE were decreased both absolutely. B-lymphocytes mostly showed only a relative increase. Successful immunosuppressive treatment usually accompanied by normalization of T-cell number and with decrease of B-cells. This differentiation of lymphocytes is an additional aid in the determination of activity of SLE and a useful index of immunosuppressive-cytostatic treatment.

Antibodies, Antinuclear↗

Thalidomide in the treatment of cutaneous lupus refractory to conventional therapy.

OBJECTIVE: We describe a prospective treatment study of thalidomide in a series of 22 patients with cutaneous lupus refractory to other treatments. METHODS: From 1992 to 1998, 22 patients with cutaneous lupus (9 with discoid lupus erythematosus, 7 with subacute cutaneous lupus, 4 with profundus lupus, 2 with nonspecific rash) were treated with thalidomide. Initial treatment was started at 100 mg daily. If the cutaneous lesions vanished, the dose was lowered to 50-25 mg daily as a maintenance therapy and it was considered a complete remission. If the lesions improved but remained, this was considered a partial response and treatment was continued until the lesions were not further modified. Periodically, adverse effects were evaluated. RESULTS: Three patients discontinued treatment because of side effects such as vertigo, persistent drowsiness, or paresthesia. Rash improved in 16/19 patients (84%). Complete remission occurred in 12/16 (75%). In 9 (65%) the rash resolved, but recurred 4-16 weeks after withdrawal of thalidomide; when it was used again, they improved. Partial response was achieved in 4/16 (25%) patients. No response occurred in 3/19 (16%). Many patients noted improvement within 2 weeks after starting thalidomide and maximum benefit was achieved within 3 months. Five of the 14 women had amenorrhea during the treatment with thalidomide. CONCLUSION: Thalidomide is effective in the treatment of cutaneous lupus refractory to other treatments. However, only some patients had a remission; the remainder relapsed when treatment was withdrawn, or required low doses of thalidomide to preserve inactive lesions. Amenorrhea was observed as a new secondary effect of thalidomide.

Adolescent↗