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Results for “Scleroderma, Localized”

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At least 721 records · Page 40Linked to original sources
PubMed1995

Antihistone antibodies in scleroderma.

BACKGROUND: Antihistone antibodies (AHA) are usually considered the serological marker of drug-induced lupus erythematosus, but recently they have been found in patients with systemic scleroderma (SSc) and morphea. OBJECTIVE AND METHODS: We determined AHA in 43 patients with SSc, 4 patients with overlap syndrome and 11 with morphea. RESULTS AND CONCLUSIONS: AHA were demonstrated in 41.8% of the SSc patients and in 36.3% of the morphea patients. Only 1 patient with overlap syndrome had AHA. Our SSc patients with AHA had frequent cardiac and renal involvements suggesting a prognostic value of AHA. In our morphea patients AHA did not correlate with clinical features.

Adult↗
PubMed1995

Extracorporeal photochemotherapy in systemic sclerosis and severe morphea.

BACKGROUND: Extracorporeal photochemotherapy (EP) is a new immune-modulating therapy which combines cytapheresis and extracorporeal irradiation of leukocytes by UVA. Recently, patients with systemic sclerosis (SS) have been treated by this method, but the efficacy of EP is still controversial. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of EP in SS and in severe morphea. METHODS: In an open study, 7 patients with SS and 2 patients with severe morphea were treated by EP and were evaluated after 6 months of treatment. No other treatments were allowed during this period. RESULTS: Except one vasovagal reaction, EP was well tolerated in all patients. The surface of the cutaneous sclerosis was unchanged in 3 of the 7 patients with SS, whereas it was aggravated in the remaining 4 patients. There was no improvement of the visceral involvement of SS in these patients. Although the duration of SS was less than 4 years in 6 out of 7 patients, we did not observe any significant benefit from EP in SS patients. One patient with generalized morphea had stopped EP after 3 months because of loss of vascular access and could not be evaluated. In the last patient with linear morphea of the upper limbs, a reduction in the number of morphea plaques was observed, and the remaining lesions were less visible after 16 months of EP. CONCLUSIONS: The present results do not corroborate those previously published with regard to the efficiency of EP in SS of recent onset. The efficacy of ECP in morphea needs further confirmation.

Adolescent↗
PubMed1995

Effects of calcitriol on fibroblasts derived from skin of scleroderma patients.

BACKGROUND: Scleroderma is a fibrotic disorder of unknown etiology that is characterized by excessive collagen synthesis and its deposition in the skin and various internal organs. OBJECTIVE: To examine whether an overproduction of extracellular matrix molecules is a result of either increased fibroblast proliferation or increased collagen synthesis. As results of clinical trials with 1,25-dihydroxyvitamin D3 (calcitriol) have suggested beneficial effect in the treatment of scleroderma patients, the effects of calcitriol on fibroblasts derived from scleroderma and normal skin has been examined as well. METHODS: Cultures of fibroblasts were established from biopsies from involved and uninvolved skin of scleroderma patients and from skin of healthy subjects, and compared with respect to proliferation, collagen synthesis and collagen lattice contraction. RESULTS: No significant differences in cell proliferation and in the extent of fibroblast-induced collagen lattice contraction have been found between scleroderma patients exhibited a disorganized growth pattern in a monolayer culture in contrast to normal fibroblasts. Collagen synthesis tends to be higher in scleroderma fibroblasts as compared with controls. Calcitriol exerted an antiproliferative and antisynthetic effect on fibroblasts, which, however, did not discriminate healthy fibroblasts from fibroblasts derived from involved or uninvolved scleroderma plaques. CONCLUSIONS: Our findings suggest that collagen accumulation may not result from increased proliferation or altered dynamic properties of fibroblasts in a scleroderma lesion but from increased collagen biosynthesis. We additionally found that calcitriol does not selectively affect scleroderma fibroblasts.

Adult↗
PubMed1995

Elevated procollagen type I carboxyterminal propeptide production in cultured scleroderma fibroblasts.

BACKGROUND: We recently reported that the serum concentration of procollagen type I carboxyterminal propeptide (P1CP) in patients with systemic sclereosis (SSc) was elevated. In the present study, we investigated collagen metabolism in in vitro cultured scleroderma fibroblasts by measuring P1CP levels in the culture medium. METHODS AND RESULTS: Spontaneous P1CP production was 4.2 times higher in fibroblast cultures from patients with SSc (n = 11) than in those from healthy controls (n = 10). P1CP production in fibroblasts derived from diffuse cutaneous SSc patients was significantly greater than that from limited cutaneous SSc patients. The serum P1CP level in SSc patients was correlated with the P1CP production of cultured fibroblasts (r = 0.815, p < 0.005). Transforming growth factor beta increased P1CP production, and gamma-interferon decreased P1CP production similarly in both SSc and normal fibroblasts. In contrast, histamine dihydrochloride increased P1CP production only in SSc fibroblasts but not in controls. CONCLUSION: These findings suggest that P1CP production in SSc fibroblasts is relevant to in vivo collagen synthesis in SSc patients.

Adolescent↗
PubMed1994

Mucin deposits in morphea and systemic scleroderma.

BACKGROUND: Though rarely reported, mucin deposition may be observed in scleroderma. OBJECTIVE: To verify the frequency of significant amounts of mucin in the biopsy specimens. METHODS: Biopsies from 20 patients with scleroderma were reviewed and stained to verify the presence of mucin. RESULTS: Mucin deposits were found in all of the 20 specimens. CONCLUSION: Mucin deposition is probably a constant feature in both morphea and systemic scleroderma. Its relevance in differential diagnosis between scleredema and scleroderma is debatable.

Adolescent↗
PubMed1993

Coexistence of morphea and systemic sclerosis.

While the association of morphea and systemic sclerosis (SSc) is considered to be a rare condition, we observed well-demarcated sclerotic skin changes indistinguishable from morphea in 9 of 135 SSc patients who visited our clinic during the last decade. We consider this rate of incidence (6.7%) to be high enough to consider morphea to be one of the skin involvements of SSc. There was a significantly (p < 0.01) higher incidence of morphea in males (3 of 5) than in females (6 of 130). Only 3 of 111 SSc patients positive for antinuclear antibody (ANA) also showed morphea, whereas 6 of 9 patients negative for ANA showed morphea (p < 0.01). Although the mechanism underlying the development of morphea in SSc patients remains unknown, our observations suggest a heterogeneous pathogenesis related to SSc gender and ANA type.

Adult↗
PubMed1990

Is there a link between dendrocytes, fibrosis and sclerosis?

In a series of fibrotic and sclerotic diseases including scars, keloids, fibromas, scleroderma and lichen sclerosus et atrophicus, we found modifications in the dendrocyte population. Dendrocytes were numerous in fibrotic diseases associated with little deposits of collagen. Conversely, they were almost absent in sclerotic diseases when the lay-down of collagen was prominent. We hypothesize that dendrocytes, by their content in factor XIIIa, may limit the accumulation of collagen in the skin.

Dendritic Cells↗
PubMed1989

Histological and rheological grading of cutaneous sclerosis in scleroderma.

We report a combined method using histology and determination of mechanical properties of skin allowing the grading of cutaneous sclerosis in morphea and acroscleroderma. Four parameters appeared useful, i.e. the evaluation of the skin thickness and of the fibrocyte density by morphometry as well as the measurement in vivo of the deformability and of the biologic elasticity of the skin. Such evaluations of the cutaneous changes occurring in scleroderma can be used to monitor therapy.

Adult↗
PubMed1989

Clinical distribution of anticentromere antibody in Japanese patients.

Serum samples from 401 subjects were screened for the presence of anticentromere antibody using HEp-2 cells as the substrate for an indirect immunofluorescence method. Anticentromere antibody was found in 16 cases; 8 out of 62 patients with systemic sclerosis, 3 out of 7 patients with primary Raynaud's disease, 2 out of 41 patients with systemic lupus erythematosus, 1 out of 54 asymptomatic relatives of systemic sclerosis and 2 out of 50 patients with miscellaneous diseases. In systemic sclerosis, the patients with anticentromere antibody were limited in CREST (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactylia and telangiectasia) variant. As reported previously by many investigators, anticentromere antibody is considered as a useful immunologic marker for CREST variant of systemic sclerosis, although this antibody is widely distributed in other conditions with less frequency.

Autoantibodies↗
PubMed1980

Circulating immune complexes in systemic scleroderma and generalized morphea.

There is growing evidence that pathologic changes in the vascular system are implicated in the pathogenesis of systemic scleroderma. It has been suggested that immune complex deposition may be responsible for such changes. We measured circulating immune complexes in 10 patients with severe systemic scleroderma, 1 of whom had clinical evidence of renal disease, and in 3 patients with generalized morphea. None of the patients had significantly elevated levels. Our findings suggest that although circulating immune complexes are of diagnostic and prognostic value in other collagen vascular diseases, they do not play a major role in the pathogenesis of systemic scleroderma in patients who lack clinical evidence of renal disease.

Adult↗
PubMed1979

Morphaea-like cutaneous changes in a patient with systemic scleroderma.

A case of systemic scleroderma with peculiar morphaea-like lesions is reported. The patient at first started with typical acrosclerosis, some parts of which later became softened and apparently resumed the appearance of normal skin, so that morphaea-like cutaneous plaques which remained hard, were soft. These changes were surrounded by erythema resembling the 'lilac ring' as noted in characteristic active lesions of morphaea.

Female↗
PubMed2001

Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients.

Patients with scleroderma receiving Iloprost as a treatment for severe Raynaud's phenomenon report a reduction in skin tightness, suggesting that this drug inhibits skin fibrosis. Connective tissue growth factor (CTGF), a recently described profibrotic cytokine, acts downstream and in concert with TGF-beta to stimulate the fibrotic process and is involved in the fibrosis seen in scleroderma. Here we show that Iloprost, acting by elevation of cAMP, blocks the induction of CTGF and the increase in collagen synthesis in fibroblasts exposed to TGF-beta. The potency of Iloprost with respect to suppression of CTGF far exceeds that of other prostanoid receptor agonists, suggesting that its effect is mediated by the prostacyclin receptor IP. By sampling dermal interstitial fluid using a suction blister device, we show that CTGF levels are greatly elevated in the dermis of scleroderma patients compared with healthy controls and that Iloprost infusion causes a marked decrease in dermal CTGF levels. These studies suggest that Iloprost could be reducing the level of a key profibrotic cytokine in scleroderma patients and that endogenous production of eicosanoids may limit the fibrotic response to TGF-beta.

Cells, Cultured↗
PubMed2001

Von Willebrand factor propeptide as a marker of disease activity in systemic sclerosis (scleroderma).

In 44 consecutive patients with systemic sclerosis (SSc), plasma concentrations of von Willebrand factor (vWf) were higher than those of the vWf propeptide, but the propeptide showed less variability within patient subgroups. Higher values of the propeptide were observed in patients with early pulmonary involvement. A closer correlation of the propeptide than of vWf to biochemical markers of activity was also evident. Our results suggest that the propeptide, despite a shorter circulating half-time and lower plasma concentrations than vWf, is more useful in the assessment of disease activity in SSc.

Adult↗