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Results for “Scleroderma, Localized”

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[Systemic sclerosis (scleroderma) without scleroderma].

There are two approaches toward dealing with systemic sclerosis (scleroderma) presenting with no skin involvement. According to one of these the condition is to be treated as atypical systemic sclerosis (scleroderma). With the other approach it does not make much difference whether systemic sclerosis presents with skin involvement or not. In the study made a group of systemic sclerosis patients presenting with no skin involvement was examined vs patients with circumscribed skin involvement. No evidence has been found for systemic sclerosis (scleroderma) sine scleroderma to be regarded as running an atypical course.

Adolescent↗
PubMed2001

Clinical significance of soluble CD31 in patients with systemic sclerosis (SSc): association with limited cutaneous SSc.

OBJECTIVE: To determine serum levels of soluble CD31 (sCD31) and its clinical associations in patients with systemic sclerosis (SSc). METHODS: Serum sCD31 levels from 70 patients with SSc were examined by ELISA. For a longitudinal study, 64 sera from 17 SSc patients were analyzed (followup: 0.4-3.9 yrs). RESULTS: Serum sCD31 levels were elevated in patients with SSc (n = 70) compared with healthy controls (n = 20) and patients with systemic lupus erythematosus (n = 15). Serum sCD31 levels were higher in patients with limited cutaneous SSc (lSSc; n = 37) than those with diffuse cutaneous SSc (n = 33). Patients with elevated sCD31 levels had pulmonary fibrosis and decreased percentage vital capacity (%VC) less frequently than those with normal sCD31 levels. sCD31 levels correlated positively with %VC in patients with SSc. This association of elevated sCD31 levels with the lower frequency of pulmonary involvement and better %VC was still observed when analyzed among ISSc patients alone. The elevation of sCD31 was associated with shorter disease duration in patients with lSSc. In a longitudinal study, 75% of patients with SSc showed increased sCD31 levels only transiently in the early phase of the disease. Serum sCD31 levels remained normal during followup in all patients with normal sCD31 levels at the first visit. CONCLUSION: Elevated sCD31 levels were associated with ISSc with relatively early onset and lower frequency and severity of pulmonary fibrosis. These results suggest that sCD31 would be a protective factor for the development of skin sclerosis and pulmonary fibrosis in SSc, since sCD31 has an antiinflammatory effect by inhibiting CD31 mediated transendothelial migration of leukocytes.

Adolescent↗
PubMed2002

Ventricular late potentials in systemic sclerosis: relationship with skin involvement.

OBJECTIVE: To detect the myocardial involvement in patients with systemic sclerosis (SSc) by signal averaged electrocardiography method (SA-ECG) in relationship to the skin changes. METHODS: We selected 24 SSc outpatients according to American Rheumatism Association criteria, without clinical and instrumental evidence of cardiac disease, and compared them with 24 control subjects. All patients and controls underwent SA-ECG to detect ventricular late potentials (LP). The extent and severity of skin involvement in SSc patients was detected by modified Rodnan (m-Rodnan) skin score. RESULTS: SSc patients had higher prevalence of LP compared to controls (46 versus 8%, p < 0.003). Median skin score value in the overall SSc population was 7 [mean, SD (range): 7.8, 3.2, (4-18)]. Patients with LP had a higher median skin score value compared to SSc patients without LP [median (range): 10 (6-18) versus 6 (4-9); Mann-Whitney U test 22.5, p < 0.005]. A subset analysis was also performed to verify the correlation of antibodies positivity (anti-centromere and Scl 70) pattern and the presence of LP. Our findings showed that higher values of skin score correlated with the presence of LP independent of antibody subsets. CONCLUSIONS: Our data suggest that diffuse abnormalities of the cardiac tissue detected by SA-ECG may be present, even in patients without cardiac symptoms. The relationship between LP and skin involvement in patients without clinical evidence of cardiac involvement may help detect of a subset of patients who may develop scleroderma heart disease.

Adult↗
PubMed2002

Causes of death and poor survival prognostic factors in thai patients with systemic sclerosis.

Causes of death and poor prognostic factors for patients with systemic sclerosis (SSc) were studied in 222 cases. Their mean age at the onset and duration of disease was 48.9 +/- 12.0 years and 23.3 +/- 29.3 months, respectively. Fifty-three per cent were diffuse subtype. Patients with diffuse SSc had more digital pitting scars and more muscle, heart, lung, and esophageal involvement than those with limited subtypes (p < or = 0.02). One hundred and six patients were lost to follow-up. With a median follow-up duration of 25 months, 31 of the remaining 116 patients (26.7%) died. SSc related death occurred in 18 cases, in which the lung, heart and kidney (renal crisis) were the major causes. Infection contributed to the remaining 13 deaths. When compared with living patients, using a univariate analysis, factors associated with a reduced survival rate were age of > 45 years at the onset, diffuse skin thickness, and lung, gastrointestinal tract, heart, kidney and muscle involvement (p < or = 0.001). In the multivariate analysis, only age of > 45 years at onset and cardiac involvement remained poor prognostic factors (p = 0.04 and 0.001, respectively).

Adult↗
PubMed2003

Scleroderma.

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Activities of Daily Living↗
PubMed2003

[Eosinophilic fasciitis--current knowledge].

First time eosinophilic fasciitis (EF) was described by Shulman. It occurrences predominantly in young men, rarely older women, sporadic in children. The illness was characterised by swelling of the extremities, skin changes, myalgia, and elevation of the peripheral blood eosinophil count and hypergammaglobulinemia. It is followed by rapidly progressive cutaneous and subcutaneous induration. The histopathologic examination in the region of affected skin shows thickening of the fascia, deep dermal fibrosis and infiltration mainly consists of lymphocytes, plasmocytes, eosinphils. It is suggested that the cells from infiltration induce fibrosis. The tryptophan disturbance has significant influence on development of EF in predisposed persons. Eosinophilic fasciitis is classified to wide spectrum of scleroderma disorders by many authors. The authors tried to show differences between EF, morphea and systemic scleroderma.

Autoimmune Diseases↗