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At least 19 recordsLinked to original sources
PubMed1985

Enhanced platelet adhesion to collagen in scleroderma. Effect of scleroderma plasma and scleroderma platelets.

The effect of plasma on platelet adhesion to collagen coated microtiter wells was investigated in 22 patients with scleroderma and 26 control subjects. In the control subjects, platelet adhesion was 38 +/- 13% (mean +/- SD) of adhesion with buffer alone; in scleroderma patients adhesion was 64 +/- 20% (p less than 0.001). No correlation was seen between the effect of plasma on platelet adhesion to collagen and the plasma levels of either FVIII/von Willebrand factor antigen or fibronectin in either scleroderma or control subjects. Furthermore, scleroderma platelets demonstrated enhanced adhesion compared to control platelets when tested in the presence of either control or scleroderma plasma.

Blood Vessels↗
PubMed1996

Expression of interleukin-4 in scleroderma skin specimens and scleroderma fibroblast cultures. Potential role in fibrosis.

BACKGROUND: Scleroderma (systemic sclerosis) is a fibrotic disease characterized by an uncontrolled tissular accumulation of collagen. Several cytokines have been implicated in the fibroblast activation leading to fibrosis. For instance, we have previously demonstrated that interleukin-4 (IL-4) is a potent activator of collagen synthesis in fibroblast cultures. In this study, using immunocytochemical methods and in situ hybridization, we investigated the expression of IL-4 in normal and scleroderma skin and fibroblast cultures. OBSERVATIONS: Immunocytochemical studies with anti-IL-4 antibody were performed on biopsy specimens from 9 patients with normal skin and 11 patients with scleroderma. The label was intense or strong in 8 of the 11 scleroderma skin specimens, whereas it was negative or faint in 8 of the 9 normal skin specimens (P < .01). In situ hybridization demonstrated a significant increase of the number of IL-4 messenger RNA grains in scleroderma skin compared with normal skin (3.1 +/- 1.5 [mean +/- SD] vs 0.8 +/- 0.7; P < .001). A strongly positive labeling with the anti-IL-4 antibody was found in the 4 scleroderma fibroblast cultures, whereas it was negative in the 5 fibroblast control cultures (P < .05). CONCLUSIONS: Our results demonstrate that IL-4 is strongly expressed in the dermis of a large majority of patients with scleroderma and might be synthesized by scleroderma fibroblasts. We suggest that IL-4 is one of the cytokines implicated in the early steps of the fibrotic process.

Adult↗
PubMed1995

Sample size calculations in scleroderma: a rational approach to choosing outcome measurements in scleroderma trials.

Subjects with both diffuse and limited scleroderma were studied to calculate the baseline characteristics of several commonly used outcome measurements in order to provide parameters for sample size calculations for scleroderma clinical trials. From these estimates, outcome measurements were chosen as potentially responsive to change in clinical trials if their sample sizes were not prohibitively large. Forty-five patients with scleroderma were systematically assessed to determine the means and standard deviations whereby sample size calculations can be performed using this information. Examples of sample sizes were determined for the entire group, and for 2 subsets: those with diffuse scleroderma and those with diffuse disease of recent onset. Many baseline characteristics were significantly different in patients with diffuse compared to limited systemic sclerosis. The baseline values were different for the Health Assessment Questionnaire (HAQ) disability score, Functional Index, grip strength, oral aperture, finger-to-palm distance, skin score, and physician global assessment. Sample sizes can vary widely depending upon the outcome measurement chosen and the range of deltas used within the different scleroderma subsets. Sample size requirements for many outcome measures are extremely large due to marked variability in the baseline measures. Primary outcome measures in scleroderma trials should be chosen which have adequate power to detect a minimal clinically relevant change in the primary outcome measurements at the sample sizes employed. All other outcome measures should be ranked as secondary. Skin scores, global assessments, and grip strength measurements require smaller sample sizes than the other outcome measurements which were studied. Sample sizes in future trials will vary depending upon the proportion of patients with diffuse and limited scleroderma who are included.

Female↗
PubMed2002

Keloid morphea and nodular scleroderma: two distinct clinical variants of scleroderma?

BACKGROUND: For nearly a century, the terms "keloid morphea" and "nodular scleroderma" have been used interchangeably without defined clinical or histologic criteria. OBJECTIVE: To define the conditions "keloid morphea" and "nodular scleroderma" by correlating the clinical and histologic features. METHODS: We retrospectively identified six patients with keloidal lesions and nodules from 70 consecutive patients with scleroderma seen in the dermatology clinic. The clinical presentation and histopathological findings were reviewed. RESULTS: Six of 70 patients with scleroderma (45 systemic and 25 morphea) exhibited keloidal or nodular lesions. All these patients had systemic sclerosis. Clinically one patient (case 1) had nodules; five (cases 2-6) had keloids. The nodular lesions had histologic findings consistent with keloid, while the keloidal plaques were variably keloids or morphea histopathologically. There was no correlation between the clinical morphology and the histologic findings, except for cases 5 and 6. These patients were African-American, with a family history of keloids and typical keloids clinically and histologically that developed from sites with normal skin. CONCLUSION: Keloid morphea and nodular scleroderma are clinical terms that describe keloidal and nodular lesions in patients most commonly with scleroderma. The clinicopathologic association is variable. Based on a review of the English literature and our series of six patients, we identified two clinical variants; (1) keloidal or nodular lesions arising from sclerodermatous skin with histologic findings of keloid or scleroderma, (2) typical keloids clinically and histologically, arising in normal skin in patients with a family history of keloids. Awareness of these entities is important for proper diagnosis of the cutaneous lesions and for recognizing that the cutaneous findings may be a sign of systemic sclerosis

Adolescent↗
PubMed1986

Antinuclear antibodies and anti-DNA antibodies in scleroderma. A possible relationship between joint manifestations and increased antibodies in localized scleroderma.

Antinuclear antibodies (ANA), including anti-DNA antibodies, and rheumatoid factors (RAT, Waaler-Rose) were determined prospectively during a 3-year period in 40 patients with localized scleroderma (LS) compared with 77 patients with generalized scleroderma (GS). ANA were increased in 26% of patients with LS, and in 47% with GS, anti-DNA antibodies in 23% of patients with LS, and in 34% with GS. Thus, the anti-DNA antibody level was lower compared with the known level in systemic lupus erythematosus. Rheumatoid factors were present in 6-7% of patients with LS, and in 14-15% of patients with GS. Increased antinuclear antibodies were not associated with any specific type of localized scleroderma, nor with internal disorders, and no case of clinical overlap to discoid or systemic lupus erythematosus was observed. However, six patients with localized scleroderma and complaints of arthralgia all presented increased antibodies, and one patient showed overlap to rheumatoid arthritis. It is suggested that increased ANA and anti-DNA antibodies in localized scleroderma, associated with joint manifestations, represents a systemic component in this type of scleroderma, with activation of the immune system and similarities with generalized collagen diseases.

Antibodies, Antinuclear↗
PubMed1996

Localized scleroderma in breast cancer patients treated with supervoltage external beam radiation: radiation port scleroderma.

BACKGROUND: An association between systemic scleroderma, radiation, and breast cancer has been recognized. However, localized scleroderma in the radiation port of breast cancer patients has been rarely described. OBJECTIVE: Our purpose was to describe the concurrence of localized scleroderma, supervoltage radiation, and breast carcinoma. METHODS: Patients were prospectively evaluated in a tertiary care cancer center, and the literature was reviewed. RESULTS: We describe six patients with breast cancer in whom localized scleroderma developed within their radiation port. CONCLUSION: Radiation port localized scleroderma can be a sequela of supervoltage radiation therapy in patients with breast cancer. Recognition is important because localized scleroderma can clinically mimic recurrent breast carcinoma.

Breast Neoplasms↗
PubMed1988

[Linear circumscribed scleroderma--case report. Classification and differentiation of circumscribed scleroderma].

In linear scleroderma, a rare form of circumscribed scleroderma, the lesions are arranged in a band-shaped linear distribution and both the superficial and the deeper layers of the skin are attached to the underlying structures. This disease must principally be differentiated from eosinophilic fasciitis (Shulman's syndrome). On the basis of the clinical and laboratory data, systemic scleroderma can be excluded in nearly all the cases. Nevertheless, patients with linear scleroderma might develop systemic scleroderma or other systemic diseases of the connective tissue even after years. As a consequence, thorough physical examination as well as laboratory evaluation is necessary over a long follow-up period. We introduce a new classification of circumscribed scleroderma.

Adult↗
PubMed1998

Increased expression of TGF-beta receptors by scleroderma fibroblasts: evidence for contribution of autocrine TGF-beta signaling to scleroderma phenotype.

Scleroderma fibroblasts exhibit numerous phenotypic differences when compared with healthy skin fibroblasts. Some of these differences, in particular overexpression of collagen type I and other extracellular matrix proteins, parallel the effect of transforming growth factor-beta (TGF-beta) on dermal fibroblasts, suggesting that the scleroderma fibroblast phenotype may result from activation of autocrine TGF-beta signaling. To test this hypothesis we examined the role of TGF-beta Type I and Type II receptors in regulating collagen type I transcription. We have shown that overexpression of either Type I or Type II receptors significantly (3-4-fold) increases alpha2 (I) collagen promoter activity in transient transfection assays in dermal fibroblasts. Addition of anti-TGF-beta antibody abolished, whereas addition of plasmin enhanced, the stimulatory effect of receptor overexpression on collagen promoter activity, suggesting that this effect depends on autocrine TGF-beta. Moreover, these cotransfection experiments indicated that expression levels of TGF-beta receptors is a limiting factor in the autocrine regulation of collagen type I transcription by TGF-beta. Comparison of the TGF-beta receptor Type I and Type II mRA expression levels in scleroderma and normal fibroblasts have indicated elevated expression (2-fold) of both receptor types in scleroderma cells, which correlated with increased binding of TGF-beta. Significantly, elevated TGF-beta receptor levels correlated with elevated alpha2 (I) collagen mRNA levels. These results suggest that the elevated production of collagen type I by scleroderma fibroblasts results from overexpression of TGF-beta receptors.

Collagen↗
PubMed1992

[Systemic scleroderma and scleroderma-like disease after silicone implants].

Systemic sclerosis or clinical patterns indistinguishable from systemic sclerosis have previously been demonstrated in workers in the polyvinylchloride industry and in mine-workers exposed to silica dust. In recent years, an increasing number of cases of systemic sclerosis, localized scleroderma, and scleroderma-like disease have been diagnosed in women after implantation of silicone gel prosthesis either following operations for breast cancer or cosmetic augmentation mammoplasty. We present two cases of systemic sclerosis or scleroderma-like disease appearing after silicone implants. Together with the already reported cases these results should lead to reduced use of silicone for cosmetic augmentation mammoplasty and a search for another material for patients operated on for breast cancer. The cases described, together with the reports concerning industrially provoked scleroderma, are of interest for the continued efforts to clarify the pathogenesis of scleroderma. Other types of exposure than those described here may also be of interest, i.e. occupational exposure to silicone spray.

Female↗
PubMedDate not supplied

[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↗
PubMed1999

Sympathetic skin response in scleroderma, scleroderma overlap syndromes and inflammatory myopathies.

Sympathetic skin response (SSR), a non-invasive method for evaluation of the autonomic nervous system, was studied in 57 patients with various connective tissue disorders: scleroderma, dermatomyositis, polymyositis, scleromyositis and unclassified collagenoses. The patients were divided into three main groups: scleroderma (SSc), myositis or other inflammatory myopathy (M) and scleromyositis (ScM). The aim of the study was to detect abnormalities of the SSR in the connective tissue diseases, to define the pattern for each group and to evaluate the usefulness of SSR in detection of subclinical impairment of sympathetic cholinergic function. In the myositis group, an abnormal SSR was found in 88% of patients; the main abnormality was absence of the response from the lower limbs (in 50% of patients). In scleroderma, the SSR was abnormal in 77% of patients, consisting mainly of absence of the response from the lower limbs, whereas responses from the upper limbs were normal. In scleromyositis, the SSR was abnormal in 80% of patients, the most frequent finding was an increase in latency in one limb. The SSR changes were most pronounced in connective tissue disorders with myositis or inflammatory myopathy. The SSR, although non-disease-specific, because of its sensitivity, seems to be useful in the assessment of the abnormalities of the autonomic nervous system in scleroderma and inflammatory myopathies. The study showed a very high prevalence of autonomic nervous system dysfunction in connective tissue diseases associated with myopathy or myositis, displaying no clinical symptoms of autonomic system involvement.

Adolescent↗
PubMed1998

Cocaine-induced scleroderma and scleroderma renal crisis.

Scleroderma renal crisis is a clinical syndrome of systemic sclerosis that is classically characterized by accelerated hypertension, rapidly progressive renal failure, and hyperreninemia. It has been reported that cocaine may initiate scleroderma in an already susceptible individual or unmask it at an earlier age in subclinical disease. We describe a 46-year-old male patient with a history of heavy cocaine abuse that unmasked scleroderma and precipitated scleroderma renal crisis.

Cocaine-Related Disorders↗
PubMed1985

Rectal prolapse in scleroderma: case report and review of the colonic complications of scleroderma.

Scleroderma of the colon is commonly associated with constipation, as was the case in a 70-year-old woman with rectal prolapse described by the authors. The chronic constipation in this patient may have been the cause of her rectal prolapse, but the onset of the prolapse and scleroderma at about the same time suggest that the scleroderma may have been a causative factor. A Ripstein repair of the prolapse was carried out. The authors discuss some of the complications of colonic scleroderma, which include megacolon, transverse and sigmoid colonic volvulus, telangiectasia, stenosis and diverticula and stercoral ulceration.

Aged↗
PubMed1990

[Classification of circumscribed scleroderma. A multicenter study of 286 patients. The Scleroderma Study Group of the Society of Dermatologic Research].

In a multicentre study performed by the German Working Group for Dermatological Research a total of 286 female and male patients with circumscribed scleroderma (morphoea) were examined with reference to form and extent of their skin manifestations (e.g. progressive idiopathic atrophodermia, Pasini-Pierini Type) and systemic involvement. On the basis of the literature and our observation of circumscribed scleroderma classification into three types appears to be appropriate: a plaque-type (with generalized circumscribed scleroderma as the most severe variant), a linear type (with pansclerotic disabling morphoea as the most severe variant) and a profound type (with eosinophilic fasciitis as the most severe variant).

Adolescent↗