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Results for “Scleroderma, Localized”

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PubMed2003

Evaluation of medium-dose UVA1 phototherapy in localized scleroderma with the cutometer and fast Fourier transform method.

PURPOSE: To evaluate the efficacy of medium-dose UVA1 phototherapy in patients with localized scleroderma. METHOD: A controlled pilot study with medium-dose UVA1 (48 J/cm2) was performed. The results were evaluated by means of a skin score and two objective methods for quantifying sclerosis (cutometer and fast Fourier transform method). Patients were treated 4 times a week for 5 weeks. The follow-up period was 12 weeks. RESULTS: All patients responded to therapy. Skin score and cutometer results showed improvement of skin elasticity of treated skin compared to control skin. Fast Fourier transform measurements showed no change in bundle orientation ratio and spacing. CONCLUSION: We concluded that treatment for 12 weeks 4 times a week with medium-dose UVA1 may be a beneficial therapy and a well-tolerated treatment modality for localized scleroderma (morphea). After 12 weeks, improvement of skin sclerosis can be detected by skin score and cutometer measurements but not by the fast Fourier transform method.

Adolescent↗
PubMed2003

Serum levels of tumor necrosis factor and interleukin-13 are elevated in patients with localized scleroderma.

BACKGROUND: The enhanced production of some cytokines may be related to the pathogenesis of localized scleroderma (LSc). OBJECTIVE: To determine whether serum levels of tumor necrosis factor (TNF) and interleukin (IL)-13 are elevated, and whether they correlated with clinical or serological features in patients with LSc. METHODS: Serum levels of TNF and IL-13 were examined by ELISA in 45 patients with LSc and in 20 healthy controls. RESULTS: The frequency of serum TNF detection was significantly higher in patients with LSc (24%) compared with that in healthy controls (0%). The frequency of serum IL-13 detection was also significantly higher in patients with LSc (29%) compared with that in controls (0%). In patients with LSc, elevated TNF levels significantly correlated with the presence of IgM antihistone antibodies, anti-single-stranded DNA antibodies, elevated serum IL-6 levels, the number of linear lesions, and muscle involvement. Elevated IL-13 levels were significantly associated with the number of plaque lesions and the total number of lesions. CONCLUSIONS: These results suggest that TNF and IL-13 may be associated with the development of LSc.

Adolescent↗
PubMed1988

Flow cytometry of fibroblasts cultured from skin of patients with localized scleroderma.

Fibroblast cultures were initiated from affected and unaffected skin sites of 6 patients with localized scleroderma. Two of the affected cell lines exhibited more than threefold increases in procollagen production and mRNA levels. All the cell lines were analyzed by flow cytometry to detect a possible heterogeneity of scleroderma fibroblast cultures which could explain the production of excessive amounts of collagen. No evidence of a subpopulation responsible for elevated collagen production was detected using cytoplasmic dot hybridization of cells fractionated by flow cytometry. When compared with the nonaffected controls, all the cell lines from affected skin areas of scleroderma patients were found to exhibit a lower level of cellular autofluorescence, suggesting an alteration in metabolic activity. The results show that the heterogeneity of scleroderma fibroblasts that was found in vivo is lost when the cells are cultured.

Adolescent↗
PubMed1988

Identification of fibroblasts responsible for increased collagen production in localized scleroderma by in situ hybridization.

Skin biopsies from seven patients with localized scleroderma (morphea) and from two healthy individuals were studied by in situ hybridization to localize the cells responsible for increased procollagen production. In scleroderma lesions, high levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs were detected in some but not all fibroblasts, suggesting the presence of a subpopulation responsible for the increased collagen production. The levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs in these fibroblasts were clearly elevated compared to control skin specimens hybridized at the same time under identical conditions. Most of the scleroderma samples represented intermediate stages where the fibroblasts containing elevated levels of type I and type III procollagen mRNAs were located in the papillary and upper reticular layer of the dermis. One of the scleroderma samples from an early inflammatory stage of the disease was found to contain activated fibroblasts in all dermal layers and also in aggregates adjacent to inflammatory cell infiltrates. In situ analyses were also performed on cell cultures from affected and unaffected skin of one scleroderma patient. These experiments revealed a homogeneous population of activated fibroblasts in cultures producing high levels of collagen. The results suggest that development of fibrosis in scleroderma could evolve through activation of a certain fibroblast subpopulation. During cell culturing, however, cell selection or uncharacterized regulatory mechanisms appear to modulate the behavior of these cells with respect to collagen production.

Autoradiography↗
PubMed2003

Thirteen-megahertz ultrasound probe: its role in diagnosing localized scleroderma.

BACKGROUND: Ultrasound imaging has been shown to be useful for the evaluation of systemic and localized scleroderma (LS). However, its specificity and sensitivity have not been studied. OBJECTIVES: To define morphological ultrasound diagnostic criteria in LS and to test their sensitivity and specificity with a 13-MHz ultrasound probe. METHODS: Forty plaques in 26 consecutive patients with LS were examined and compared blindly with 17 control plaques in 16 patients with skin diseases where LS was in the differential diagnosis. Data were also compared with a normal control group. Five patients were re-evaluated 12-18 months after the first examination. RESULTS: Ultrasound examination disclosed a characteristic dense image resembling a flattened 'yo-yo'. Undulations of the dermis, disorganization, loss of thickness and thickened hyperechoic bands in the hypodermis, and the 'yo-yo' image had a high sensitivity and a high specificity for LS. A 92% sensitivity and a 100% specificity for LS were found when at least four of these five signs were present. CONCLUSIONS: Thirteen-megahertz ultrasound is a valuable tool for diagnosing LS. Morphological ultrasound diagnostic criteria had a high specificity and a high sensitivity.

Adolescent↗
PubMed2006

Treatment of pediatric localized scleroderma with methotrexate.

OBJECTIVE: To analyze the effectiveness of methotrexate (MTX) for the therapy of pediatric localized scleroderma (LS). METHODS: A retrospective chart review was performed for 17 pediatric patients with LS who failed topical therapy and were subsequently treated with MTX (12.5-25 mg weekly) with or without oral corticosteroids. A structured followup telephone call to the families was used to assess patient satisfaction. RESULTS: Skin findings improved in 16 of 17 patients with a median time to improvement of 2.25 and 2.0 months for MTX alone or in combination with corticosteroids. Only one patient had active lesions at the most recent followup visit. Fifteen of 17 families reported improvement in their child's lesions after beginning MTX. Twelve of 17 patients were treated with MTX and oral corticosteroids. There were no major adverse events. CONCLUSION: MTX appears to be a safe and effective therapy for pediatric LS.

Adolescent↗
PubMed1978

Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.

Adolescent↗
PubMed1993

Localized scleroderma progressing to systemic disease. Case report and review of the literature.

We describe a 15-year-old girl with biopsy-proven morphea who developed progression to systemic disease 2 years after initial presentation. In contrast to other reported patients with localized scleroderma, some of whom have had mild, nonprogressive systemic involvement, this patient developed severe, debilitating disease, with skin tightness, sclerodactyly, esophageal involvement, restrictive pulmonary disease, and myopathy. From the time of her initial evaluation, the patient was positive for antinuclear antibodies (ANA), which were shown to be primarily directed against the Ku antigens. This observation suggests that ANA may be a prognostic indicator for progression to systemic disease.

Antibodies, Antinuclear↗
PubMed1989

Serum aminoterminal propeptide of type III procollagen in progressive systemic sclerosis and localized scleroderma.

Sera from 31 patients with progressive systemic sclerosis (PSS), 5 patients with widespread localized scleroderma (LS), and 3 patients with lichen sclerosus et atrophicus were analyzed for aminoterminal propeptide of type III procollagen (PIIINP) using a radioimmunoassay based on human propeptide. Thirty-eight per cent of the patients with PSS had levels above normal range, including all of the 3 patients with diffuse scleroderma. The same applies to 4 of 5 patients with widespread localized LS, while PIIINP in all 3 patients with lichen sclerosus et atrophicus were within normal levels. In patients with acrosclerosis, elevated PIIINP seems to be correlated to rapid progression and extension of lesions. A significant increase in PIIINP was found in a patient following discontinuation of prednisone and cyclophosphamide, while the present investigation did not allow judgement of effects of treatment with either penicillamine or cyclosporin A.

Humans↗
PubMed2000

Increased serum levels of soluble vascular cell adhesion molecule 1 and E-selectin in patients with localized scleroderma.

BACKGROUND: The serum levels of soluble forms of vascular cell adhesion molecule 1 (sVCAM-1) and E-selectin (sE-selectin) can be used as a marker of endothelial activation. OBJECTIVE: We investigated whether the serum level of sVCAM-1 and sE-selectin in patients with localized scleroderma (LSc) was correlated with the clinical or serologic features of this disease. METHODS: Serum samples from 59 patients with LSc, 20 patients with systemic sclerosis (SSc) and 29 healthy controls were examined using specific enzyme-linked immunosorbent assays. RESULTS: The serum levels of sVCAM-1 and sE-selectin in patients with LSc were significantly higher than those of the healthy controls. The serum levels of sVCAM-1 and sE-selectin were correlated with both the number of sclerotic lesions and the number of involved areas. CONCLUSION: These results indicate that the serum levels of sVCAM-1 and sE-selectin may reflect the extent of the skin involvement (ie, the disease severity) and may be useful for monitoring the in vivo states of endothelial activation in LSc.

Adult↗
PubMed2006

A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma.

BACKGROUND: In previous trials, UV therapy has been demonstrated to be effective in the treatment of localized scleroderma (LS). To date, a randomized comparison study to evaluate the efficacy and safety of different, commonly used phototherapeutic modalities in LS is still outstanding. OBJECTIVE: The aim of this study was to compare the safety and efficacy of low-dose (LD) UVA1, medium-dose (MD) UVA1, and narrowband (NB) UVB phototherapy in the treatment of LS. METHODS: Sixty four patients with LS were consecutively included in a prospective, open, randomized controlled 3-arm study. Severity of LS was determined by means of a clinical score, and clinical improvement was also monitored by histopathologic analysis and 20-MHz ultrasound. RESULTS: A total of 27 patients were treated with LD UVA1 (20 J/cm2), 18 patients received MD UVA1 (50 J/cm2), and 19 patients were treated with NB UVB dependent on their skin type. Phototherapy was performed 5 times weekly for 8 weeks. Two of the 64 patients included in this trial discontinued therapy. Skin status significantly improved in all patients who finished the treatment protocol, resulting in a reduction of the clinical score in all groups (LD UVA1, 7.6-5.0 [P < .001, 95% confidence interval 1.6-3.4]; MD UVA1, 11.1-6.6 [P < .001, 95% confidence interval 2.5-6.2]; NB UVB, 7.3-4.9 [P < .001, 95% confidence interval 1.6-3.2]). The reduction of the score was accompanied by an improvement of the visual analog scale for itching and tightness, histologic score, and 20-MHz ultrasound. MD UVA1 was significantly more effective than NB UVB (P < .05). There were no significant differences between LD UVA1 and NB UVB and the former and MD UVA1 (P > .05). LIMITATIONS: We had a relatively small study sample and nonblinded assessment of primary outcome. CONCLUSION: Phototherapy, as previously reported in several noncontrolled trials, is an effective therapeutic option in LS, with a favorable risk/benefit ratio. UVA1 phototherapy should be considered among the first approaches in the management of LS.

Adolescent↗
PubMedDate not supplied

Clinical significance of serum matrix metalloproteinase-13 levels in patients with localized scleroderma.

OBJECTIVES: To investigate the clinical significance of serum matrix metalloproteinase-13 (MMP-13) levels in patients with localized scleroderma (LSc). METHODS: Serum MMP-13 levels were determined by using a peptide substrate cleavage assay in 10 patients with generalized morphea, 10 with linear scleroderma, 10 with morphea, and 10 normal controls. RESULTS: The serum MMP-13 levels in patients with LSc were lower than those in normal controls, but there was no significant difference (64.9 +/- 19.9 versus 73.2 +/- 11.5, p = 0.058). Serum MMP-13 levels in patients with generalized morphea were significantly lower than those in normal controls (54.0 +/- 18.7 versus 73.2 +/- 11.5 ng/ml; p < 0.01). Serum levels of MMP-13 were comparable among normal controls, the patients with linear scleroderma, and those with morphea. The prevalence of muscle involvement was significantly greater in the LSc patients with decreased MMP-13 levels compared with those with normal MMP-13 levels (50% versus 8%, p < 0.05). Serum MMP-13 levels were significantly inversely correlated with the number of linear lesions (r = 0.366, p < 0.05) and the number of involved body areas (r = 0.552, p < 0.005) in patients with LSc, while there was no significant correlation between serum MMP-13 levels and the number of plaque lesions. Furthermore, there was significant inverse correlation between serum MMP-13 levels and the number of involved body areas in patients with generalized morphea (r = 0.631, p < 0.05). CONCLUSION: The serum MMP-13 levels may reflect the disease severity in patients with LSc, especially generalized morphea, the severest form of this disorder.

Adolescent↗
PubMed2001

Localized scleroderma or morphea?

Morphea is a frequently mild, benign, and self-limiting skin disease with a less than 1% reported chance of progressing to systemic scleroderma. Morphea is a sufficient and less terrifying name for these disorders than localized scleroderma.

Humans↗
PubMed1996

Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid, and other fibrotic skin disorders.

Connective tissue growth factor (CTGF) is a novel peptide that exhibits platelet-derived growth factor-like activities and is produced by skin fibroblasts after activation with transforming growth factor-beta. Coordinate expression of transforming growth factor-beta followed by CTGF during wound repair suggests a cascade process for control of tissue regeneration. We recently reported a significant correlation between CTGF mRNA expression and histologic sclerosis in systemic sclerosis. To confirm the relation between CTGF and skin fibrosis, we investigated CTGF gene expression in tissue expression in tissue sections from patients with localized scleroderma, keloid, other sclerotic skin disorders using nonradioactive in situ hybridization. In localized scleroderma, the fibroblasts with positive signals for CTGF mRNA were scattered throughout the sclerotic lesions with no preferential distribution around the inflammatory cells or perivascular regions, whereas the adjacent nonaffected dermis was negative for CTGF mRNA. In keloid tissue, the fibroblasts positive for CTGF mRNA were diffusely distributed, especially in the peripheral expanding lesions. In scar tissue, however, the fibroblasts in the fibrotic lesions showed partially positive signals for CTGF mRNA. In eosinophilic fasciitis, nodular fasciitis, and Dupuytren's contracture, CTGF mRNA was also expressed partially in the fibroblasts of the fibrotic lesions. Our findings reinforce a correlation between CTGF gene expression and skin sclerosis and support the hypothesis that transforming growth factor-beta plays an important role in the pathogenesis of fibrosis, as it is the only inducer for CTGF identified to date.

Adolescent↗
PubMedDate not supplied

Localized scleroderma after infection with Epstein-Barr virus.

Over a period of three years (1989-1992) five children suffering from localized scleroderma were seen at the Department of Pediatrics of the University of Trieste. Evidence of a previous infectious mononucleosis (IM) was present in four out of five patients. The clinical history of these four children is reported. The association between the appearance of scleroderma and a previous viral infection is not surprising. However, in the pediatric literature there is only one case of progressive systemic sclerosis (PSS) developing in a 15-month-old girl less than one month after she contracted IM. The presence of shared epitopes between an Epstein-Barr virus protein, BOLF1, and the hypervariable region of HLA associated with the pauciarticular form of JCA, recently reported, could provide a key to the pathogenesis of other collagen diseases such as scleroderma.

Adolescent↗
PubMed1997

The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993.

OBJECTIVE: To determine the incidence, prevalence, survival rates, clinical manifestations, and longterm outcome of patients with morphea (localized scleroderma) and its subtypes over a 33 year period in Olmsted County, Minnesota. METHODS: We used the unique data resources of the Rochester Epidemiology Project to review all Olmsted County medical records with any potential diagnosis consistent with morphea (including plaque, generalized, bullous, linear, and deep entities) from 1960 through 1993. RESULTS: We screened 1030 medical records and identified 82 (59 female; 23 male) cases of morphea first diagnosed between 1960 and 1993. All cases were followed until death or migration from Olmsted County, a total of 754 person-years of observation. The annual age and sex adjusted incidence rate per 100,000 population was 2.7 (95% confidence interval 2.1, 3.3). The incidence rate increased significantly over the 33 years (p = 0.0037) on an average of 3.6% per year. The prevalence (estimated using cumulative incidence) at 80 years of age was about 2/1000. 50% of the patients had a cutaneous softening or evidence of disease resolution by 3.8 years' duration. The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 years) compared to 5.5 years in the deep group. Arthralgias, synovitis, uveitis, and joint contractures were more frequent in the linear and deep categories. Although 9 patients (11%) developed some disease related disability over the followup period, this was common (44%) in the deep group. No case of morphea developed severe internal organ involvement and none progressed to systemic sclerosis. The survival rate was not significantly different from the general population (p = 0.409). CONCLUSION: Morphea, and its subtypes, are more common than previously recognized, and can lead to important disability.

Adolescent↗
PubMed1993

Localized scleroderma associated with Borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations.

BACKGROUND: Recent reports have implicated Borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). OBJECTIVE: Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. METHODS: Ten patients were examined clinically and by routine microscopy. Biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. RESULTS: Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4+ subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. Patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. CONCLUSION: Our findings suggest that some cases of LS are linked to Borrelia infection.

Adolescent↗