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Results for “Scleroderma, Localized”

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PubMed1995

[Cold-induced cardiac abnormalities in systemic sclerosis].

Cardiac abnormalities are frequent in patients with systemic sclerosis (SS). These abnormalities have been demonstrated in over 80% of patients with SS and there are some clues that suggest that an intermittent vascular spasm (i.e. coronary Raynaud's phenomenon) is one of the causes of myocardial dysfunction in this group of patients. The aim of this prospective study was to evaluate the ventricular performance and regional wall motion during exposure to cold in patients with SS and Raynaud's phenomenon without overt cardiac disease. Twenty-four patients and 10 normal volunteers underwent radionuclide ventriculograms (RV). In each subject the RV was done thrice: basal, 20 minutes after chilling with thermic blanket and post reheating. The left ventricular ejection fraction (LVEF) decreased during the cold test (p = 0.03) with reversible abnormalities in wall motion. Basal Right Ventricular Ejection Fraction (RVEF) was lower than that of normal subjects (p = 0.02) and decreased during the cold test (p = 0.04). Therefore, we were able to demonstrate an early cardiac dysfunction associated with impaired wall motion after corporal chilling. These findings suggest that coronary spasm in SS would be an early and frequent phenomenon that would precede the development of symptomatic cardiac disease in some patients with this illness.

Adult↗
PubMed1998

[Silicone and autoimmune diseases].

There exists controversy surrounding the potential relationship between silicone implants and autoimmune diseases. Indeed, silicone is not an inert substance and microparticles from the prosthesis can migrate causing localised or generalised reactions. Most notably, silicone has been associated with sclerodermia and human adjuvant disease. Sclerodermia has been observed within 50% patients suffering from autoimmune diseases linked with silicone. For patients with symptoms, the removal of the prosthesis achieves a favourable response in 50% of the cases. Before a patient undergoes a silicone implant, it is essential that personal and family history of autoimmune disorders are ascertained and evaluated. Also, the patient should undergo regular check ups after having received the implant. In spite of the fact that certain epidemiological studies have not demonstrated a relationship between silicone implants and autoimmune disease, the continual appearance of new cases means that the physician should have an open attitude and be aware of the possibility.

Autoimmune Diseases↗
PubMed1998

Autoantibodies against calpastatin in sera from patients with systemic sclerosis.

OBJECTIVE: To determine the prevalence and clinical correlation of anti-calpastatin antibodies in patients with systemic sclerosis (SSc). METHODS: Serum samples from patients with limited cutaneous SSc (lSSc; n=36), diffuse cutaneous SSc (dSSc; n=27), and healthy control subjects (n=29) were examined by an ELISA using human recombinant calpastatin as antigens. RESULTS: IgG anti-calpastatin antibodies were positive in 15 (24%) of 63 patients with SSc, which was similar to the frequency of IgM anti-calpastatin antibody positivity (14/63, 22%). The presence of anticentromere antibodies was associated with the presence of IgG and/or IgM anti-calpastatin antibodies. The patients with SSc positive for IgG anti-calpastatin antibodies had significantly higher erythrocyte sedimentation rates (ESR) compared with those negative for IgG anti-calpastatin antibodies. Furthermore, the levels of IgG anti-calpastatin antibodies correlated positively with the levels of ESR. The patients with dSSc positive for IgM anti-calpastatin antibodies had significantly higher frequency of inflammatory joint and muscle involvement compared with those negative for IgM anti-calpastatin antibodies. Four (80%) of 5 patients with dSSc with both joint and muscle involvement produced IgM anti-calpastatin antibodies. CONCLUSION: The presence of anti-calpastatin antibodies may be a clue to mechanisms of the inflammatory change occurring in SSc. Furthermore, the presence of anti-calpastatin antibodies could be a novel and useful serologic tool for recognizing patients with dSSc with inflammatory joint and muscle involvement.

Adolescent↗
PubMedDate not supplied

Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension.

Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and is generally an early finding. The present study was conducted to investigate whether quantitative nailfold capillaroscopy could distinguish those limited scleroderma patients who have established pulmonary hypertension. Quantitative nailfold capillaroscopy was performed by Visual Image Analysis in 10 healthy subjects and 20 patients with limited scleroderma (18 centromere +ve), of whom 8 had established pulmonary hypertension. It was found that scleroderma patients with pulmonary hypertension had a significant reduction in capillary density compared with patients lacking this complication (p < 0.01). Patients with scleroderma have significantly more dilated capillaries than controls although no significant differences were observe between the two patient subgroups. The finding of reduced nailfold capillary density in scleroderma patients with established pulmonary hypertension has possible pathogenic significance and may allow detection of this subgroup at an early stage in their disease progression.

Adolescent↗
PubMed1998

Unilateral linear lichen sclerosus et atrophicus.

We report on a 23-year-old woman with unilateral lichen sclerosus et atrophicus (LSA) who exhibited extensive eruptions on the right extremities and right aspect of the abdomen. The lesions on the right extremities appeared to exhibit a linear pattern along Blaschko's lines, while the right abdominal skin lesion was a whitish, parchment-like plaque. Histological findings for both lesions showed characteristic features of LSA. LSA should be differentiated from the linear type and the macular type of localized scleroderma.

Abdomen↗
PubMed1998

Iloprost as cyclic five-day infusions in the treatment of scleroderma. An open pilot study in 20 patients treated for one year.

OBJECTIVE: To evaluate the safety and efficacy of cyclic intravenous iloprost therapy in diffuse or limited scleroderma. METHODS: Twenty patients, 14 women and 6 men with a mean age of 47.8 +/- 8.2 years, were given iloprost infusions for six hours a day during five consecutive days, at a rate of 0.5 to 2 ng/kg/min. The course was repeated every three months for one year. Efficacy was evaluated based on a scleroderma skin lesion score, an ischemic lesion score, a well-being self-assessment score, and lung function tests including measurement of the diffusing capacity of the lung for carbon monoxide. Safety was assessed based on adverse event collection. RESULTS: The scleroderma skin lesion and ischemic lesion scores decreased significantly over the one-year treatment period, from 37.1 +/- 16.5 to 10.2 +/- 6.9 (P < 0.001) and from 31.8 +/- 19.1 to 2.2 +/- 2.0 (P < 0.05), respectively. The well-being self-assessment score also showed a significant improvement, from 71.4 +/- 16.5 to 15.0 +/- 6.6 (P < 0.001). The diffusing capacity for carbon monoxide was decreased in 11 patients at baseline and showed a slight, non significant increase in these patients after the treatment period. No serious or persistent side effects were recorded. CONCLUSION: Cyclic intravenous iloprost therapy was associated with improvements in skin changes and in general health, as well as with a slight increase in the diffusing capacity of the lung for carbon monoxide. Our data suggest that iloprost may act on some of the pathogenetic mechanisms of scleroderma in a way that improves the course of the disease.

Female↗
PubMed1978

Collagen in the cellular and fibrotic stages of scleroderma.

The collagen in localized and systemic scleroderma skin was studied by light microscopy with silver impregnation (50 patients), electron microscopy (14 patients), and immunofluorescence microscopy using specific antibodies against Type I and Type III collagens (12 patients). In the cellular stage, the dermis and adipose tissue revealed perivascular or diffuse cellular infiltrates (mostly lymphocytes, plasma cells, and macrophages), accompanied by deposition of Type III collagen. The lower dermis also showed an increase in Type III collagen. In the fibrotic stage, the papillary layer showed a reduction and/or clumping of Type III collagen as compared to normal skin. The lower dermis and the adipose tissue revealed compact collagen consisting exclusively of Type I collagen or a mixture of Type I and Type III collagen. The pattern of Type III collagen distribution was similar to that of reticulin, thus suggesting that at least some reticulin fibrils may represent Type III collagen.

Adipose Tissue↗
PubMed2004

Hyperplasia of dermal microvascular pericytes in scleroderma.

BACKGROUND: Pericytes (PCs) are smooth muscle-like mural cells of capillaries and venules, which can synthesize matrix components and fibroblast-activating cytokines, and are thus potential mediators of pathological changes in scleroderma. In this study, alterations in microvessels were quantitatively imaged, taking PC into account for the first time. METHODS: Skin biopsies from systemic (12) and localized (14) scleroderma forms as well as age-, sex-, and body location-matched controls were examined with respect to capillary and venular densities as well as endothelial cell (EC) and PC counts using a newly developed (in respect of PC and EC) indirect collagen IV immunostaining-based method. RESULTS: Hyperplasia of the PC that doubled the microvascular PC density was the most conspicuous characteristic. In the capillaries of the upper dermal plexus of the periphery of the sclerotic zones, median ratios of PC : EC were 0.23 (controls 0.10) or 0.18 (controls 0.11) in systemic or localized scleroderma, respectively. Furthermore, an increase in capillary density in the upper dermal plexus could be demonstrated in the marginal zones of both types of disease. CONCLUSIONS: The observed PC increase in the peripheral zones of active disease supports the hypothesis of a vascular pathogenesis of scleroderma and directs the focus to microvascular PC.

Adolescent↗
PubMed2002

Bullous lesions in scleroderma.

BACKGROUND: The occurrence of bullous lesions in localized or systemic scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases. OBJECTIVE: To investigate the frequency, clinical, and immunopathologic features of patients with scleroderma and bullous eruptions and to review the literature regarding this rare condition. METHODS: A retrospective study of 53 cases of scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases. RESULTS: Four of 53 patients exhibited bullous lesions in association with scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic scleroderma. CONCLUSIONS: Of the 53 original patients, we have described four cases of bullous scleroderma (7.5%) Illustrating several pathogenetic mechanisms of bulla formation. inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying scleroderma.

Adult↗
PubMedDate not supplied

[Acupuncture in treatment of inflammatory rheumatic diseases].

Seventeen studies were examined with regard to efficacy and scientific quality of acupuncture in rheumatoid arthritis, spondarthropathy, lupus erythematosus, local and progressive systemic scleroderma. Acupuncture cannot be recommended for treatment of these diseases. By far, the most studies examined failed to show sufficient quality.

Acupuncture Therapy↗
PubMed2000

Analysis of dendritic cell populations using a revised histological staging of morphoea.

BACKGROUND: Recent studies have suggested that dermal dendritic cells (DDCs) may play a part in maintaining the structure of the dermis and in dermal immune modulation. Alteration in the population of DDCs has been noted in localized and systemic scleroderma, particularly a decline in the number of CD34+ DDCs. Objectives To define the alteration of the DDC populations with respect to the histological stage of morphoea. METHODS: We examined 33 biopsies of morphoea, categorized into four histological stages, and examined the DDC population (CD34+ DDCs and factor XIIIa+ DDCs), the lymphocytic infiltrate, and tenascin (extracellular matrix glycoprotein) and transforming growth factor (TGF)-beta1 expression in each biopsy. RESULTS: As the dermis became less inflammatory and more sclerotic, there was a significant decline in the number of CD34+ DDCs and an increase in the number of factor XIIIa+ DDCs. The pan-T-cell infiltrate (UCHL-1/CD45RO) and tenascin deposition exhibited a similar pattern, with elevated expression in inflammatory stages and a decrease in expression as the dermis became sclerotic. TGF-beta1 was significantly elevated in three of the four histological stages of morphoea, in both the inflammatory and sclerotic stages. The proposed four-stage histological analysis of morphoea biopsies was a useful basis for studying dendritic cells and mediators in cutaneous sclerosis. CONCLUSIONS: Our study indicates that there is a reciprocal relationship between CD34+ DDCs and factor XIIIa+ DDCs in morphoea that correlates with the relative degrees of inflammation and sclerosis.

Adolescent↗