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Results for “Scleroderma, Localized”

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PubMed1978

Scleroderma.

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Adult↗
PubMed1984

B-cell markers in malignant B-cell lymphoma with scleroderma-like manifestation.

A case is described of malignant B-cell lymphoma with scleroderma-like manifestation. Using different monoclonals as B-cell markers the tumor appeared to be positive for surface immunoglobulins (SmIg) and for B2-antigen, but negative for intracytoplasmic immunoglobulin (CIg), BA2- and FMC7-antigens. Therefore, the tumor could be determined as a highly differentiated Sm-positive early B-cell type of B-cell lymphoma. In this clinically rare manifestation of cutaneous B-cell lymphoma aspects of the cell morphology and of cellular mediated immunity are briefly discussed.

Aged↗
PubMed2005

Photodynamic therapy in dermatology--an update.

Topical photodynamic therapy (PDT) is a well-established treatment modality which has mainly shown to be effective for dermatooncologic conditions like actinic keratoses (AK), Bowen's disease, in situ squamous cell carcinoma and superficial basal cell carcinoma (BCC). However, a therapeutical benefit of PDT is also evident for inflammatory dermatoses like localized scleroderma, acne vulgaris and granuloma annulare. Recent work has been focused on the development and evaluation of topical photosensitizers like the heme precursor 5-aminolevulinic acid (5-ALA) or its methyl ester (methyl aminolevulinate) inducing photosensitizing porphyrins. These drugs do not induce strong generalized cutaneous photosensitization like the systemically applied porphyrins or their derivatives. For dermatological purposes, incoherent lamps or light-emitting diode arrays can be used for light activation. Depending on the applied light dose and the concentration of the photosensitizer either cytotoxic effects resulting in tumor destruction or immunomodulatory effects improving the inflammatory conditions occur. Treating superficial oncologic lesions (tumor thickness <2-3 mm) cure rates achieved by PDT are equal to the cure rates of the respective standard therapeutic procedure. The benefits of PDT are the low level of invasiveness and the excellent cosmetic results after treatment.

Dermatology↗
PubMed1975

Serum copper and caeruloplasmin in ankylosing spondylitis, systemic sclerosis, and morphea.

Studies of serum copper and caeruloplasmin were performed in patients with ankylosing spondylitis, systemic sclerosis, and morphea. Mean levels of both were raised significantly in ankylosing spondylitis, with the greatest increases in the worst cases. In patients with systemic sclerosis there was a significant increase in the mean level of caeruloplasmin, but not of copper, although both were raised in the 2 patients with the most aggressive disease. No alterations were found in patients with morphea. The values in the patients overlapped considerably with the values in the control subjects. It is thought that the increase in serum copper is probably secondary to the increase in caeruloplasmin which occurs as a nonspecific response to inflammation.

Ceruloplasmin↗
PubMed1985

Autoantibodies in childhood scleroderma.

The clinical presentation of scleroderma in childhood is even more varied than in adult life. This study of 19 children shows that antinuclear antibodies (ANA) detected on the HEp2 cell substrate are just as common as in the adult disorders. All seven children with diffuse scleroderma or acrosclerosis were ANA positive, as were eight of the 12 with more localised disease. Antinucleolar antibodies were particularly frequent (58% overall), but anticentromere antibody was not observed. Enzyme-linked immunosorbent assays (ELISAs) for antibodies to collagen types I-V, in contrast to adult scleroderma, gave normal results in all childhood cases.

Antibodies, Antinuclear↗
PubMed1993

Early phenotypic activation of circulating helper memory T cells in scleroderma: correlation with disease activity.

OBJECTIVES: The differential expression of several accessory/activation molecules (CD26, CD29, CD45RA, CD25, MLR4, HLA-DR) on peripheral blood CD4+ and CD8+ T lymphocytes in patients with scleroderma was compared with that in controls and patients with other connective systemic diseases to look for evidence of the involvement of T cells in the disease process of scleroderma. METHODS: The two colour expression of surface molecules by circulating T cells was analysed with a panel of monoclonal antibodies and flow cytometry in 17 patients with scleroderma, 10 patients with systemic lupus erythematosus, and five patients with rheumatoid arthritis, and the results compared with those for 10 normal controls. The two colour T CD4+ phenotype was further compared between patients with active and quiescent disease in these patients with scleroderma. The coexpression of surface molecules by CD4+ T cells was also analysed by three colour flow cytometry in eight patients with scleroderma. RESULTS: Patients with scleroderma showed increased CD4+CD26+ and CD4+CD25+ percentages and absolute numbers and decreased CD8+CD29+ percentages compared with controls. Moreover, a significant correlation between the higher CD4+CD26+ T cell percentage and absolute cell numbers with disease activity was observed. Most of the CD4+ peripheral blood T cells from patients with scleroderma showed the CD26+CD45RA- phenotype by three colour flow cytometry analysis. CONCLUSIONS: The distinctive pattern of early helper memory T cell activation in these patients with rapidly evolving scleroderma supports the role of a T cell mediated mechanism in the progression of scleroderma.

Adult↗
PubMed2003

Risk of cancer in patients with scleroderma: a population based cohort study.

BACKGROUND: Previous studies have suggested an increased risk of cancer among patients with scleroderma. OBJECTIVE: To study a population based cohort of patients with scleroderma in South Australia. METHODS: Subjects with scleroderma were identified from the South Australian Scleroderma Registry established in 1993. All subjects on the scleroderma registry were linked to the South Australian Cancer Registry to identify all cases of cancer until 31 December 2000. Standardised incidence ratios (SIRs) for cancer for subjects with scleroderma were determined using the age- and sex-specific rates for South Australia. RESULTS: In 441 patients with scleroderma, 90 cases of cancer were identified, 47 of which developed after inclusion on the scleroderma registry. The SIRs for all cancers among these patients were significantly increased (SIR=1.99; 95% confidence interval (95% CI) 1.46 to 2.65) compared with the cancer incidence rates for South Australia. The SIRs for lung cancer (SIR=5.9; 95% CI 3.05 to 10.31) were also significantly increased. The SIRs for all cancers among the subgroups with diffuse scleroderma (SIR=2.73; 95% CI 1.31 to 5.02) and limited scleroderma (SIR=1.85; 95% CI 1.23 to 2.68) were significantly increased. CONCLUSIONS: This population based cohort study provides evidence that scleroderma is associated with cancer, and in particular, lung cancer. In addition, both diffuse and limited forms of scleroderma are associated with a similarly increased risk of cancer.

Adolescent↗
PubMed2003

Antiphospholipid antibody in localised scleroderma.

OBJECTIVE: To investigate the prevalence and clinical correlation of antiphospholipid antibodies in localised scleroderma. METHODS: Antibodies against cardiolipin (aCL) or beta(2)-glycoprotein I were examined by enzyme linked immunosorbent assay (ELISA) in 48 patients with localised scleroderma (18 patients with generalised morphoea, 20 with linear scleroderma, and 10 with morphoea). Twenty one of these patients were investigated for lupus anticoagulant (LAC) by screening and confirmatory coagulation tests. RESULTS: Patients with generalised morphoea, the severest form of localised scleroderma, had significantly raised levels of IgM or IgG aCL relative to normal controls (n=21) and patients with systemic sclerosis (n=20). The IgM isotype was predominant, with the frequency of IgM aCL (61%) higher than that of IgG aCL (28%). Levels of aCL were similar for patients with linear scleroderma or morphoea and normal controls. IgM aCL were associated with a greater number of lesions, especially plaque lesions, wider distribution of lesions, and the presence of immunological abnormalities including antinuclear antibodies, rheumatoid factor, IgM antihistone antibodies, IgG anti-single stranded DNA antibodies, and raised serum interleukin 6 levels in patients with localised scleroderma. LAC was detected in 5/7 (71%) patients with generalised morphoea. However, pulmonary embolism was seen in only one patient with generalised morphoea. None of patients with localised scleroderma exhibited anti-beta(2)-glycoprotein I antibodies. CONCLUSIONS: These results suggest that aCL and LAC are the major autoantibodies in patients with generalised morphoea.

Adult↗