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Results for “Scleroderma, Localized”

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At least 217 records · Page 12Linked to original sources
PubMed2005

Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma.

OBJECTIVE: To evaluate the efficacy of pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy in patients with severe localized scleroderma (LS). DESIGN: A prospective, nonrandomized, open pilot study. SETTING: Dermatology department at a university hospital in Bochum, Germany. Patients Fifteen patients with histologically confirmed severe LS. Interventions Oral methotrexate (15 mg/wk) combined with pulsed intravenous methylprednisolone (1000 mg for 3 days monthly) for at least 6 months. MAIN OUTCOME MEASURES: Treatment outcome was evaluated by means of a clinical score, 20-MHz ultrasonography, and histopathologic analysis. Safety assessment included the monitoring of adverse effects and clinical laboratory parameters. RESULTS: One patient discontinued therapy. In most of the remaining 14 patients, significant elimination of all signs of active disease (inflammation) and remarkable softening of formerly affected sclerotic skin that resulted in a decrease of the mean +/- SD clinical score from 10.9 +/- 5.3 at the beginning to 5.5 +/- 2.5 at the end of therapy was observed (P < .001). Clinical improvement was confirmed by histologic and ultrasonographic assessments. No serious adverse effects were noted. CONCLUSIONS: These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed.

Administration, Oral↗
PubMed2002

Juvenile-onset localized scleroderma activity detection by infrared thermography.

OBJECTIVE: The aim of this study was to define the clinical utility of infrared thermography in disease activity detection in localized scleroderma (LS). METHODS: We retrospectively reviewed 130 thermal images of 40 children with LS and calculated the sensitivity and specificity of thermography, comparing clinical descriptions of the lesions and contemporary thermographs. The reproducibility of thermography was calculated by using the weighted kappa coefficient to determine the level of agreement between two clinicians who reviewed the thermographs independently. RESULTS: The sensitivity of thermography was 92% and specificity was 68%. Full concordance between the two clinicians was observed in 91% of lesions, with a kappa score of 0.82, implying very high reproducibility of this technique. CONCLUSION: Our results demonstrate that thermography is a promising diagnostic tool when associated with clinical examination in discriminating disease activity, as long as it is applied to lesions without severe atrophy of the skin and subcutaneous fat. Further evaluation is needed to determine whether thermography can predict the future progression of lesions.

Age of Onset↗
PubMed1998

Vitamin K1-induced localized scleroderma (morphea) with linear deposition of IgA in the basement membrane zone.

We describe a 45-year-old white man in whom distinctive clinical and histologic features of localized scleroderma developed at sites of injection of vitamin K1 (phytonadione). A direct immunofluorescence test demonstrated prominent linear deposition of IgA along the basement membrane zone. No circulating antibasement membrane zone IgA antibodies were identified on indirect immunofluorescence testing. We believe that the unusual immunofluorescence finding in our patient is nonspecific and represents an epiphenomenon caused by cutaneous injury.

Antifibrinolytic Agents↗
PubMed1985

Total hemiatrophy. Association with localized scleroderma, Schönlein-Henoch nephritis, and paroxysmal nocturnal hemoglobinuria.

In the clinical course of a patient with progressive facial hemiatrophy associated with ipsilateral body atrophy (total hemiatrophy), signs and symptoms of localized scleroderma were noted. The patient subsequently was found to have Schönlein-Henoch purpura with renal involvement and, later, paroxysmal nocturnal hemoglobinuria. To our knowledge, such an association has not been reported before.

Adult↗
PubMed1985

Increased central cornea thickness in localized scleroderma (morphoea).

The central cornea thickness (CCT) was measured in 17 patients with localized scleroderma (morphoea) by the Haag-Streit pachymeter. Results were compared with measurements in healthy persons matched with respect to sex and age. CCT was increased (p 0.01) in patients with morphoea (mean 0.535 mm, range 0.510-0.580, SD 0.0217) as compared to the controls (mean 0.511 mm, range 0.490-0.525, SD 0.0094). In 9 (53%) of the patients CCT was more than mean + 2 SD in the controls. CCT was correlated to the duration of morphoea (correlation coefficient 0.660, p 0.01). It is discussed that the increase in cornea thickness may be a sign of minimum cornea "swelling" with alterations of the glycosaminoglycans of the corneal stroma as a possible background. The study demonstrates that morphoea is not simply a local disease confined to the plaques of the skin.

Adolescent↗
PubMed1986

Assessment of epidermal atrophy in localized scleroderma (morphea).

The skin surface of scleroderma skin becomes glossy as a result of epidermal atrophy. In this study, the skin relief was studied in 12 patients with localized scleroderma (morphea). Measurements by a stylus method showed smoothening of the skin in all plaques studied (p less than 0.01). Ultrasound measurement showed increased skin thickness (p less than 0.01). There was no correlation between smoothening of the skin surface and increase in skin thickness, and no correlation to the duration of the plaques. It is concluded that glossy appearance of plaques of scleroderma is an early sign, which may be useful in the clinical diagnosis of scleroderma.

Adult↗
PubMed2001

Localized scleroderma in a 12-year-old girl presenting as gingival recession. A case report and literature review.

Scleroderma is a connective tissue disorder that displays considerable clinical heterogeneity. This case describes a 12-year-old girl who presented with a localized form of the disease. The consequences were a severe and progressive localized gingival recession affecting two maxillary incisors, a localized lip defect and scarring of the forehead. The case illustrates the difficulties in diagnosis and management of young patients with localized scleroderma.

Child↗
PubMed2005

Follow-up efficacy of integrative Chinese and Western drugs on localized scleroderma with vitamine B6 and Xuefu Zhuyu decoction.

OBJECTIVE: To investigate the therapeutic effects of vitamine B(6) (Vit B(6)) and Xuefu Zhuyu Decoction (XFZY, for activating blood circulation to remove stasis) in patients with localized scleroderma(LSD). METHODS: Thirty-three patients were treated with XFZY and Vit B(6), with 15 cases taking orally prednisone acetate and 20 healthy volunteers as the control. Their level of soluble interleukin-2 receptor (sIL-2R) and tumor necrosis factor-alpha (TNF-alpha) in the patients with LSD before and after treatment were observed. RESULTS: The level of sIL-2R and TNF-alpha in the serum from the patients with LSD were higher than those of healthy volunteers (P < 0.01). After treatment with Vit B(6) and XFZY, the level of sIL-2R and TNF-alpha from the patients with LSD decreased significantly (P < 0.01), but there were no difference between the group taking Vit B(6) plus XFZY and the group given prednisone. CONCLUSION: The activating blood circulation to remove stasis approach in treating LSD with integrative Chinese and Western drugs got better results, and metabolic disorder of tryptophan might be correlated with the etiology of LSD.

Adolescent↗
PubMed2003

Comparative biochemistry of human skin: glycosaminoglycans from different body sites in normal subjects and in patients with localized scleroderma.

OBJECTIVES: The aim of this investigation is to compare the relative proportions of disaccharides of chondroitinase-digestible glycosaminoglycans (GAGs) among the different body sites in control human skin and in the skin lesions of patients with localized scleroderma. METHODS: The disaccharide relative proportions were determined using high-performance liquid chromatography (HPLC). RESULTS: DeltaDi-4S, the main disaccharide unit of dermatan sulphate (DS), was the major skin GAG disaccharide (approximately 70% of the total) in control skin among all different body sites studied here. In scleroderma there was an increase in the relative proportion of both deltaDi-HA, the main disaccharide unit of hyaluronic acid (HA), and deltaDi-diS(B) (alpha-deltaUA(2SO4)-1-->3-GalNAc(4SO4)), derived from DS, and a decrease in deltaDi-4S, as compared with the uninvolved skin or the site-matched control skin. CONCLUSION: DS is the major GAG species in normal skin from different body sites. In addition, our results suggest a decrease and also a structural change in DS and an increase in the proportion of HA in scleroderma skin.

Adult↗
PubMed1995

Clinical aspects of systemic and localized scleroderma.

After the skin, the gastrointestinal tract is the most frequently affected organ in systemic sclerosis. Gastrointestinal symptoms already may be present early in the course of the disease and do not necessarily correlate with objective findings. Esophageal dysmotility is not specific for systemic sclerosis but occurs in other connective tissue diseases as well. Peripheral macrovascular disease was shown to be increased in patients with limited cutaneous sclerosis; signs of autonomic dysfunction were found in patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) variant. Pulmonary involvement was shown to be moderately or severely decreased in 40% of a large cohort of scleroderma patients. In one study, no support was found for the association between pulmonary involvement and gastroesophageal reflux. Peripheral nerve involvement is often subclinical and might be associated with anti-U1-RNP and anti-topoisomerase I antibodies. Internal organs are seldomly affected in localized scleroderma. When occurring in childhood and involving an extremity, localized scleroderma can cause growth failure, resulting in long-term functional disability.

Humans↗
PubMed1975

Localized scleroderma.

Familial scleroderma is rare; only seven documented instances of the disease have been reported, to our knowledge. This report adds two more families to the literature. Three children in one family and two in the other had clinically and histiologically established localized scleroderma.

Adolescent↗
PubMed1999

Localized scleroderma associated with progressing ischemic stroke.

We present a 73 year-old Japanese woman with localized scleroderma involving the right side of the scalp accompanied by continuous tingling pain, who developed insidiously progressive left hemiparesis. In magnetic resonance imaging of the brain, an infarct first appeared in the watershed region of the right middle cerebral artery territory and subsequently extended to deep white matter accompanied by scattered hemorrhages. Focal stenosis in the M2 portion of the right middle cerebral artery was revealed on magnetic resonance angiography, and the distal vessels were only shown faintly. A biopsy specimen from the sclerotic scalp lesion showed obvious thickening of vessel walls and mild mononuclear cell infiltration. We believe that the progressing ischemic stroke was caused by hemodynamic disturbances from localized sclerotic obstruction of the middle cerebral artery, with an autoimmune pathogenesis.

Aged↗
PubMed1992

No evidence for a spirochaetal origin of localized scleroderma.

We looked for evidence of a Borrelia infection in 15 patients with morphoea. We were not able to detect antibodies to Borrelia burgdorferi in any of these 15 patients. None of the 14 skin biopsies examined by immunohistochemistry showed evidence of spirochaetes. Skin biopsies were cultured in 10 patients. All were negative. These results do not support a spirochaetal origin of localized scleroderma.

Adolescent↗
PubMed1994

Localized scleroderma after exposure to organic solvents.

A 26-year-old female developed plaques characteristic of morphea on the volar surfaces of the forearms and on the dorsal surfaces on the ankles following an exposure to trichloroethylene, tetrachloroethylene and other solvents by inhalation. Exposure to chemicals has been known to be important as a provoking factor of systemic sclerosis. This patient shows that exposure to solvents could provoke localized scleroderma.

1-Propanol↗
PubMed1983

Localized scleroderma 'en coup de sabre' and iridopalpebral atrophy at the same line.

A case is reported of scleroderma 'en coup de sabre' in a 13-year-old girl with atrophy of the nasal part of the iris and loss of cilia on the upper eyelid. The lesions of the front and eye were located on precisely the same line. The line did not follow the innervation fields of the cranial, peripheral or autonomous nerves. Nor did it follow the tension lines of the skin (Langer), or underlying anatomical structures. It is discussed that the predisposition to the 'coup de sabre' line seen in localized scleroderma was laid down in the mesenchyme in early foetal life before the differentiation of the anatomical structures.

Atrophy↗
PubMed1990

[Histological study on localized scleroderma].

To define stage-specific and type-specific histological findings in morphea, 21 (14 plaque, 2 linear, and 5 en coup de sabre type) patients were examined. A) Fibrotic changes; 1) Every case had the fibrotic change of various degrees. 2) Nodular sclerotic fibrosis was found in 7 cases with morphea, namely in 5 of 11 cases with a plaque type and 2 of 4 en coup de sabre type morphea. And the above mentioned 7 cases were within two years since the onset, and no nodular fibrosis was found in the morphea with the longer duration than two years in history. Nodular fibrosis was located in the middle or lower dermis adjacent to the typical sclerotic dermis. It was strongly suggested that this nodular fibrosis is as an initial change of localized scleroderma. 3) Nodular fibrosis expanded to the neighboring area and made a typical histological feature of morphea which is completely different from that of diffuse scleroderma. B) Inflammatory findings; 1) Inflammatory changes were divided into, a perivascular, a diffuse or non-perivascular, and a mixed type. 2) The pure perivascular type was found in 10, the pure diffuse type only in one, the mixed type in 6 cases, and the other 4 cases had no inflammation. 3) Marked or moderate inflammation was found in cases with short history of the disease except for one case. 4) Inflammatory cells in the morphea were mainly composed of lymphocytes and histiocytes, but occasionally of plasma cells in 11 of 17 cases. C) Pigmentary changes; Incontinentia pigmenti was found in 18 of 21 cases.

Adolescent↗
PubMed1999

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin 1, in patients with localized scleroderma.

OBJECTIVE: Serum autoantibodies to fibrillin 1, the major component of microfibrils in the extracellular matrix, recently have been reported to occur in the tight skin mouse and in patients with systemic sclerosis, but not in patients with other connective tissue diseases. This study was undertaken to determine whether antifibrillin 1 antibodies could be detected in patients with localized forms of scleroderma. METHODS: Sera from 50 patients with localized scleroderma (27 with linear scleroderma and 23 with morphea) and 51 normal controls were tested for IgG and IgM antifibrillin 1 autoantibodies, using a radioimmunoassay (RIA) and a human recombinant fibrillin 1 protein (rFbn-1). RESULTS: Both in patients with linear scleroderma and in those with morphea, mean levels of IgM and IgG binding to rFbn-1 were significantly higher than in controls. Eight patients with linear scleroderma (30%) and 6 patients with morphea (26%) had IgG autoantibodies to fibrillin 1 (rFbn-1) by RIA, compared with 3 controls (6%) (P = 0.006 and P = 0.022, respectively). No correlations between antifibrillin 1 antibodies and active skin disease or antinuclear antibody positivity were found. CONCLUSION: Autoantibodies to fibrillin 1 occur in patients with both forms of localized scleroderma (linear scleroderma and morphea). The clinical and pathogenetic significance of this autoimmune response remains to be determined.

Autoantibodies↗