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Results for “Scleroderma, Localized”

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At least 1,765 records · Page 98Linked to original sources
PubMed1985

Familial occurrence of dermatomyositis and progressive scleroderma after injection of a local anaesthetic for dental treatment.

In an 11-year-old boy severe dermatomyositis occurred a few days after injection in the jaw of a local anaesthetic of the amide type for tooth extraction. The patient's grandmother was affected by progressive scleroderma at the age of 34 years, also after injection of a local anaesthetic for dental treatment. Both cases are discussed with special reference to the immuno-genetic aspects of the origin of dermatomyositis.

Adult↗
PubMed1999

Animal model of sclerotic skin. I: Local injections of bleomycin induce sclerotic skin mimicking scleroderma.

We have established a mouse model for scleroderma induced by repeated local injections of bleomycin (BLM). Daily injection of BLM at a dose of >10 microg per ml for 4 wk induced histologic changes of dermal sclerosis, but not fibrosis, with thickened and homogenous collagen bundles and cellular infiltrates in BALB/C mice, whereas clinical signs of scleroderma were not apparent. In addition, lung fibrosis was also induced preceding the cutaneous changes. Sclerotic changes were not found in other sites of the skin distant from the injection site. Dermal sclerosis could also be induced by injecting BLM only every other day. The sclerotic changes of the dermis were sustained after ceasing BLM applications for at least 6 wk. Mast cells gradually increased in number as the sclerotic changes developed. Marked degranulation of mast cells was observed with elevated histamine release. The amount of hydroxyproline in skin was significantly increased at 4 wk of BLM treatment as compared with that in untreated or phosphate-buffered saline-treated mice. Anti-nuclear antibody was detected in serum of BLM-treated mice. Transforming growth factor-beta1 mRNA was detected at an early phase, while transforming growth factor-beta2 mRNA was strongly expressed at 4 wk when the sclerotic features were prominent. These results suggest that dermal sclerosis induced by BLM closely resembles systemic sclerosis both histologically and biochemically. Our mouse model can provide a powerful tool of inducing dermal sclerosis to examine the pathogenesis and the therapeutic approach of scleroderma.

Animals↗
PubMed1991

Expression of intercellular adhesion molecule-1 (ICAM-1) in the skin of patients with systemic scleroderma.

The expression and tissue distribution of intercellular adhesion molecule-1 (ICAM-1) in skin biopsies from 12 patients with systemic (SSc) and localized (LS) scleroderma was studied and compared to the biopsies from patients with lupus erythematosus (LE) and normal individuals. In normal human skin ICAM-1 expression was restricted to the vascular endothelium, infiltrating mononuclear cells (MNC), and to few individual keratinocytes. In the inflammatory stage of SSc, however, the expression of ICAM-1 was dramatically increased at the site of MNC infiltrates and could also be detected on fibroblast-like cells lying well apart from these infiltrates in the deep dermis. In contrast, in LS ICAM-1 was expressed mainly at the sites of MNC infiltrates. In LE ICAM-1 expression was confined to the keratinocytes, endothelial cells, and mononuclear cells in the upper parts of the dermis. Analysis of serial tissue sections from patients with SSc demonstrated also colocalization of staining of ICAM-1 around blood vessels with LFA-1-positive lymphocytes. Increased expression of ICAM-1 in the dermis of patients with SSc may represent an important mechanism by which MNC become localized and retained at a site of connective tissue inflammation, leading to the activation of fibroblasts.

Adult↗
PubMedDate not supplied

Chromosomal alterations in scleroderma.

To bring some new arguments in support of the genetic theory regarding the etiology and pathogenesis of scleroderma 12 patients (2 males and 10 females, ranging in age between 13 and 64 years) with both localized and systemic scleroderma were studied. For each patient chromosomal preparations were made in cell cultures from peripheral blood. The structural cytogenetic aberrations, observed in proportion of 81.81%, were, in the order of frequency: chromatid breakage, gaps, acentric fragments, deletions. The alterations appeared both in the systemic scleroderma and in the localized one but cannot represent a factor of prognosis for the disease. The association of Raynaud's syndrome with cytogenetic aberrations may lead to an early diagnosis of the disease.

Adolescent↗
PubMed1988

Localization of collagen mRNA in normal and scleroderma skin by in-situ hybridization.

Scleroderma is a fibrotic disease occurring in a localized or systemic form. Disturbed regulation of connective tissue metabolism plays an important role in its pathogenesis. However, until now, most of the data available were obtained from studies of fibroblasts in culture and there is considerable doubt that fibroblasts in a monolayer reflect the in-vivo situation. Using in-situ hybridization with specific antisense RNAs on frozen sections of skin, cells were detected displaying enhanced messenger RNA levels for type I and type III collagen in patients with localized and systemic scleroderma. Activated fibroblastic cells were often located near blood vessels in the deep dermis of patients with early stages of the disease and were mostly surrounded by mononuclear cells. These findings are in agreement with the concept that the interaction of fibroblasts with 'immunocompetent cells' is crucial in the initial activation of connective tissue metabolism in fibrosis.

Adult↗
PubMed2006

A case of sclerodermatous graft-versus-host disease following autologous peripheral blood stem cell transplantation.

We report a 65-year-old woman with chronic graft-versus-host disease (GVHD) who developed severely sclerotic skin on the fingers, hand and trunk following autologous peripheral blood stem cell transplantation (APBSCT). The patient had suffered from breast cancer and been treated with surgery and chemotherapy. She showed pancytopenia and was treated with APBCST. Four years after APBSCT, she developed the severe sclerotic changes on the fingers, hands, extremities and trunk. The skin biopsy showed a flattened epidermis and a proliferation of collagen bundles in the dermis. No anti-nucleolar DNA titers were detected in the serum. She was diagnosed with chronic GVHD. Despite treatment with oral prednisolone, the skin sclerotic change developed and the breast cancer recurred. She died due to pericarditis. This is a rare case of sclerodermatous GVHD following APBSCT. The serum interleukin (IL)-12 levels were examined during the treatment.

Aged↗
PubMed2003

Localized morphea: a rare adverse effect of valproic acid.

Morphea, a specific type of cutaneous sclerosis, is known in the pediatric age group, but not as an adverse effect to the antiepileptic valproic acid. We report a 14-year-old male patient with absence seizures who, after treatment with valproic acid, developed skin tightness on the fingers. A biopsy specimen revealed dermal sclerosis consistent with morphea. His symptoms resolved gradually after discontinuation of the medication. To our knowledge, this is the first patient reported to have an apparently valproic acid-induced localized morphea.

Adolescent↗
PubMedDate not supplied

[What information can digital plethysmography provide in acrosyndromes?].

Digital plethysmography records digital pulsation and measures blood flow by occlusion and perfusion pressure. The pulsatile curve is modified in vasomotor disorders of the extremities, and is delayed and flattened in obstructive arteriopathy. In Raynaud's phenomenon, the basal flow (at 22 degrees C) decreases with clinical severity, and suppression of post-ischemic blood flow by cold seems to be an attribute of Raynaud's disease. Digital perfusion pressure is usually greater than or equal to 70% of the humeral systolic pressure. When it decreases under hot conditions, organic obstructive arteriopathy is indicated; decreases due to local cooling allow quantification of hyperreactivity to cold of the digital artery.

Cyanosis↗
PubMed1989

Unusual localization of cutaneous chronic graft-versus-host disease in the radiation fields in four cases.

Chronic graft-versus-host disease (GVHD) is a very heterogeneous entity with variable clinical expression. The pathophysiology involves autoimmune disorders and features of fibrosis. Four patients transplanted for severe aplastic anaemia conditioned with cyclophosphamide and thoraco-abdominal irradiation developed skin scleroderma specifically localized in the irradiation fields. This syndrome was remarkable for its late onset and the absence of factors known to trigger chronic GVHD. It is postulated that irradiation used in this protocol may induce keratinocyte damage and trigger chronic GVHD.

Abdomen↗