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Results for “Scleroderma, Localized”

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At least 505 records · Page 28Linked to original sources
PubMed2004

Health-related quality of life in systemic sclerosis as measured by the Short Form 36: relationship with clinical and biologic markers.

OBJECTIVE: To evaluate health-related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using the Short Form 36 (SF-36) and to correlate SF-36 scores with clinical and biologic markers. METHODS: The SF-36 was administered to 24 controls and 24 SSc patients. SSc patients also were evaluated for subset (limited SSc [lSSc] and diffuse SSc [dSSc]), age, disease duration, angiotensin-converting enzyme (ACE) levels, autoantibodies, and skin and internal organ involvement. RESULTS: The physical summary score (PSS) was lower in SSc patients than in controls (P < 0.05), whereas the mental summary score (MSS) was higher in dSSc than in lSSc patients (P < 0.05). Five of 8 single SF-36 domain scores were lower in SSc patients than in controls (P < 0.05). Vitality was higher in dSSc than in controls (P < 0.001). In SSc, elder age correlated with lower PSS; low ACE levels and high skin score correlated with higher general mental health and role limitations due to physical problems, respectively (P < 0.05). Patients with heart involvement had higher scores in general health perceptions (P < 0.05). CONCLUSION: The SF-36 shows that HRQOL is impaired in patients with SSc. Higher scores in MSS and vitality in patients with dSSc and correlations of high SF-36 scores with specific organ involvement suggest that SSc patients with severe disease are more able to cope with HRQOL modification.

Adaptation, Psychological↗
PubMed1985

Dermatofibrosarcoma non-protuberans: description and report of five cases of a morpheaform variant of dermatofibrosarcoma.

Five cases of dermatofibrosarcoma are reported. All showed features typical of dermatofibrosarcoma protuberans except that in four cases, and a portion of the fifth case, no protusion of the tumor was noted clinically despite the rather advanced stage of growth of the tumor. These lesions resembled morphea or a morpheaform basal cell carcinoma clinically but could be recognized as "dermatofibrosarcoma non-protuberans" by physicians who had observed a previous case.

Adolescent↗
PubMed1994

Autoantibodies to HMG-17 nucleosomal protein in patients with scleroderma.

Autoantibodies to HMG-17, a non-histone nucleosomal protein, were found in systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) and ANA positive pauciarticular juvenile rheumatoid arthritis (JRA), but not in rheumatoid arthritis (RA). Using highly purified HMG-17 derived from porcine thymus, we tested sera from 50 patients with scleroderma for antibodies to HMG-17 by enzyme-linked immunosorbent assay (ELISA). There were 16 patients with diffuse cutaneous systemic sclerosis (dSSc) and 34 with limited cutaneous systemic sclerosis (1SSc) with age at disease onset 40.25 +/- 11.68 and 39.94 +/- 15.68 years, respectively, and disease duration 6.03 +/- 4.98 and 13.34 +/- 11.80 years, respectively (P < 0.0001). Anticentromere antibodies (ACA) were found in 65% of 1SSc patients but not in dSSc (P < 0.0001) while the prevalence of antinuclear antibodies (ANA) with other than ACA patterns did not differ in the two groups. Anti-HMG-17 antibodies occurred in 20 patients (40%), five with dSSc (31%) and 15 with 1SSc (41%). Twelve of the 20 HMG-17 positive patients were also positive for ACA (60%) but this association was not significant. No association was found between anti-HMG-17 and other antibody patterns. In conclusion, anti-HMG-17 antibodies occur in one third of scleroderma patients, do not discriminate scleroderma variants and are not associated with other autoantibodies.

Adult↗
PubMed1992

Clinical evaluation of scleroderma spectrum disorders using a points system.

We have established a new diagnostic method using a points system to evaluate patients with early scleroderma and those with scleroderma spectrum disorders (SSD). To examine the clinical usefulness of this method, it was applied to a total of 215 cases including 97 patients with scleroderma, 32 with SSD, 28 with presumed primary Raynaud's phenomenon (RP) and 58 with other connective tissue disorders (CTD). A total score was obtained for each patient as the sum of the following five factors: (1) extent of skin sclerosis (maximum, 10 points); (2) pulmonary changes (maximum, 4 points); (3) antinuclear antibodies (maximum, 5 points); (4) pattern of Raynaud's phenomenon (maximum, 3 points); and (5) nailfold bleeding (maximum, 2 points). Of the 97 scleroderma patients, 86 (89%) had 9 or more points, and of the 32 SSD patients, 28 (88%) had 5 to 8 points. In contrast, all patients with presumed primary RP and 54 of 58 (93%) patients with other CTD had 0 to 4 points. These data suggest that this diagnostic method is very useful not only for clinical evaluation of SSD, but also for the differentiation of scleroderma and SSD from other CTD and primary RP.

Connective Tissue Diseases↗
PubMed1985

Filamentous aggregates of collagen. Ultrastructural evidence for collagen-fibril degradation in situ.

Filamentous aggregates of collagen are distinct structures in the pathological dermis. These aggregates are distinguishable from fibrous long-spacing collagen (in vitro and at biopsy) and the Luse body. The aggregates are produced from dermal collagen fibrils by clostridial collagenase and culture-medium extract, which supposedly contains cellular collagenase at a neutral pH, as well as by organ cultures. In vitro experiments showed that carrageenan granuloma contains fibrous long-spacing collagen and segment long-spacing collagen. The granuloma also contains the aggregates. The aggregates were found in skin biopsies from syphilitic chancres, acrosclerotic scleroderma, morphea, mycosis fungoides, myeloid leukemia, mastocytosis and malignant melanoma. These findings indicate that the aggregates are products of the in situ degradation of collagen fibrils by some collagenolytic factor. This factor may originate in fibroblast-like cells, reticulum cells, leukemia cells, mast cells and melanoma cells.

Biopsy↗
PubMed1978

[HLA antigen frequencies in patients with progressive systemic sclerosis and morphea (author's transl)].

Forty three patients with progressive systemic sclerosis and 24 patients with morphea are typed for 30 antigens of the HLA-A and B series. There is an increase of the frequency of HLA-B8 in patients with progressive systemic sclerosis (37% vs. 20% in controls: P less than 0.001, Pcorr. = n.s.). The increase of the HLA-B8 frequency seems to be most pronounced in patients with a diffuse form of progressive systemic sclerosis. 4 out of 5 patients are HLA-B8 positive (P = 0.00672). In patients with acrosclerosis is HLA-B8 only slightly increased (32%), but the increase is greater in male patients (44%) and in patients with an early onset of the disease (36%). With the exception of HLA-A1 there is no deviation of the frequencies of any other HLA antigen tested, especially not of HLA-Aw24. The number of patients with morphea is to small for statistical evaluation.

Adult↗
PubMed1977

Immunological studies in childhood scleroderma.

The results of immunological studies on 13 children suffering from scleroderma are reported. Antinuclear antibodies with speckled pattern of fluorescence could be found in systemic sclerosis and in 3 patients with scleroderma en bandes. The same patients (except but one with PSS) demonstrated high levels of DNA antibodies. The 2 patients with PSS were found to have a very low percentage of T cells, whereas the percentage of B cells was increased. The results demonstrate the significance of autoimmunity not only in systemic sclerosis but also in focal scleroderma. Beyond this it can be presumed that immune deficiency (with a defect in cellular immunity) may be the predisposing cause of at least progressive systemic sclerosis.

Adolescent↗
PubMed1985

Collagen synthesis in scleroderma: selection of fibroblast populations during subcultures.

In progressive systemic scleroderma, excessive deposition of collagen leads to fibrosis of several tissues including the skin. It has been found that different populations of fibroblasts are present in scleroderma skin; these can be obtained by establishing cell cultures from different layers of the involved skin. Excessive overproduction of collagen was noted in primary cultures of cells obtained from deeper layers of the skin of patients in an early stage of the disease, whereas control fibroblasts did not manifest significant variations dependent on the layers of skin used to initiate the cultures. The synthesis of type-I and -III collagen was found to be altered concomitantly. The production of collagen and collagenous proteins was then followed during subcultivations of overproducing fibroblasts. In many cell strains, increased synthesis of collagen and/or non-collagenous proteins had already been lost after the first subcultivation, whereas overproduction was stable in others. However, after five passages, most of the cultures showed normal collagen synthesis, which probably indicates a loss of phenotype due to successive subcultures or overgrowth by another population of fibroblasts.

Adult↗