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PubMed2004

Neuroimaging findings in scleroderma en coup de sabre.

OBJECTIVES: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). METHODS: Patients with LScs were evaluated by high-resolution MRI and CT. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures. RESULTS: Nine patients with LScs were evaluated (five women), with ages ranging from 6 to 53 years (mean, 30.7 years). Brain CT showed bone deformities with thinning of the skull under the skin lesions in six patients. MRI scans showed focal atrophy and blurring of the gray-white matter interface localized under the skin lesion in all patients. In three patients it was associated with hyperintense signal on fluid-attenuated inversion recovery (FLAIR) and T2-weighted images. Follow-up MRI showed extension of the brain lesion in one patient; in the remaining patients, the lesion did not progress. Four of the nine patients had partial epilepsy. One had surgery for management of refractory seizures, and pathologic findings indicated a focal inflammatory process. CONCLUSION: Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Epilepsy, when present, is related to these brain lesions. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progressive.

Adolescent↗
PubMed1998

[Coincidence of scleroderma and primary biliary cirrhosis. Results of a systematic study of a dermatologic patient sample].

We present for the first time in the German dermatologic literature results of systematic investigations on the relationship between scleroderma, antimitochondrial autoantibodies and primary biliary cirrhosis (PBC). 40 patients with different clinical pictures of systemic and localized scleroderma were examined. By means of indirect immunofluorescence technique, in the sera of 5 cases (12.5%) antimitochondrial autoantibodies (AMA) could be detected. The target autoantigens for the AMA were identified as pyruvate dehydrogenase, branched-chain alpha-ketoacid dehydrogenase, alpha-ketoglutarate dehydrogenase, protein x and pyruvate dehydrogenase E-1 alpha in all 5 cases, PBC was confirmed by means a liver biopsy and endoscopic retrograde cholangioscopy. Considering the occurrence in the normal population, the prevalence of the PBC in our scleroderma collective was 8.3 x 10(2) to 2.5 x 10(3) x higher. In respect to the clinical picture of the scleroderma, we found a M2-antibody-positive PBC in 2 woman with CREST syndrome and in 1 woman with a acral-type of the progressive systemic scleroderma. CREST syndrome and coexistently M2-antibodies in the serum are a risk constellation for the development of PBC. In 2 female patients older than 50 years we observed the coincidence of disseminated plaque-like localized scleroderma and a M2-antibody-positive PBC. In our opinion this latter constellation is a specific entity. The administration of ursodeoxycholic acid (daily 15 mg/kg BW) in 3 cases led not only to dramatic improvement of the clinical symptoms of PBC as expected, but also to pronounced improvement of skin lesions of 2 patients with disseminated circumscribed and 1 patient with progressive systemic sclerosis. Thus PBC should be searched for in patients with scleroderma, especially those with CREST syndrome or widespread localized disease; it may have practical therapeutic value as well as immunological significance.

Adult↗
PubMed1987

Coordinated regulation of type I and type III collagen production and mRNA levels of pro alpha 1(I) and pro alpha 2(I) collagen in cultured morphea fibroblasts.

Fibroblast cultures were started from affected and unaffected skin areas of six patients with localized scleroderma in an active stage. The cell lines were studied for synthesis of procollagens and fibronectin by metabolic labeling with 3H-proline and for their contents of mRNAs for pro alpha 1(I) and pro alpha 2(I) collagen. For this purpose a cDNA clone for human pro alpha 1(I) collagen mRNA was constructed. The clone was identified by restriction site mapping and hybridization to the specific mRNAs. All the scleroderma fibroblast lines produced increased amounts of type I and type III collagens and fibronectin during the early passages. The cell lines gradually reduced their elevated synthesis of collagen and fibronectin to normal or near normal levels by the tenth passage. The ratios of alpha 1(I) and alpha 2(I) chains and of type I and type III collagens, and the extent of type I procollagen processing, remained relatively unchanged in all the cultures. The cellular levels of type I procollagen mRNAs were increased in all the cells exhibiting an increased synthesis of collagen. The results suggest that in localized scleroderma the fibroblasts have undergone a coordinated activation of collagen synthesis at transcriptional level.

Collagen↗
PubMed1991

Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination.

Recently, a causative association has been made between the ingestion of levotryptophan (L-tryptophan) and the eosinophilia-myalgia syndrome (EMS), a new entity manifested by peripheral blood eosinophilia, myalgias, constitutional symptoms, and cutaneous edema with fibrosis. Contaminated levotryptophan preparations produced at a single manufacturing company between October 1988 and June 1989 have been implicated in all EMS cases. In this study, we analyzed retrospectively 49 patients with cutaneous fibrosis for a history of levotryptophan use. Levotryptophan ingestion prior to the onset of their disease was reported by 11 (65%) of 17 patients with eosinophilic fascilitis (EF), two (20%) of 10 patients with localized scleroderma, and none of 22 patients with systemic sclerosis. The onset of levotryptophan-associated cutaneous disease preceded the availability of contaminated levotryptophan preparations in seven (54%) of 13 patients. One patient with levotryptophan-associated generalized morphea also had lichen sclerosus et atrophicus and acanthosis nigricans, findings not previously reported in patients with EMS. In addition, we compared the clinical and laboratory features of levotryptophan-associated EF and idiopathic EF. Myalgias, muscle weakness, paresthesias, morpheaform plaques, cutaneous ulcers, and livedo reticularis were more common in patients with levotryptophan-associated EF. We conclude that levotryptophan-associated EF and localized scleroderma were present before the presumed date of contaminated levotryptophan availability. The clinical spectrum of cutaneous fibrosis associated with the ingestion of levotryptophan includes generalized morphea and EF, which are similar though not identical to their idiopathic counterparts.

Adult↗
PubMed1998

Activation of tissue inhibitor of metalloproteinases-3 (TIMP-3) mRNA expression in scleroderma skin fibroblasts.

Excessive accumulation of fibrillar collagens is a hallmark of the cutaneous fibrosis in both systemic and localized scleroderma. Turnover of the collagenous extracellular matrix is dependent on the balance between collagenolytic matrix metalloproteinases and their specific inhibitors. We have examined the expression of the novel, matrix associated tissue inhibitor of metalloproteinases-3 (TIMP-3) in normal and scleroderma skin fibroblasts in culture and in vivo. The levels of TIMP-3 mRNA were elevated up to 2.5-fold in five of seven systemic sclerosis fibroblast strains, whereas TIMP-1 mRNA expression was elevated up to 1.8-fold in two and TIMP-2 mRNA expression up to 1.8-fold in two systemic sclerosis strains. Using in situ hybridization, TIMP-3 mRNA was detected in seven of 12 localized scleroderma skin samples, specifically in fibroblasts within fibrotic collagen fibers or in the vicinity of inflammatory cells. TIMP-1 mRNA was detected in three of eight scleroderma skin samples in fibroblasts adjacent to inflammatory cells. The expression of TIMP-3 mRNA by systemic sclerosis and normal skin fibroblasts was enhanced to a similar extent (by 8.6- and 8.1-fold, respectively) by transforming growth factor-beta, and suppressed down to 34 and 54%, respectively, by tumor necrosis factor-alpha. Specific activation of TIMP-3 gene expression in scleroderma skin fibroblasts in culture and in vivo suggests a role for TIMP-3 in the pathogenesis of dermal fibrosis via inhibition of turnover of fibrotic dermal extracellular matrix, possibly due to upregulation of TIMP-3 expression by transforming growth factor-beta.

Adult↗
PubMed1996

Localized forms of scleroderma, including morphea, linear scleroderma, and eosinophilic fasciitis.

Under the term localized scleroderma a spectrum of conditions is classified, ranging from localized plaques of morphea of cosmetic importance only, to deep lesions of linear scleroderma and eosinophilic fasciitis, which can result in considerable morbidity. The etiology is unknown; environmental, infectious, and autoimmune causes have been proposed. In the past year, a revised classification of morphea has been presented. Additional information relating to pathogenesis, laboratory studies, and associated manifestations is reviewed.

Borrelia burgdorferi Group↗
PubMed1977

Failure of dimethyl sulfoxide in the treatment of scleroderma.

Nineteen patients with systemic scleroderma and five with localized scleroderma were treated with topical dimethyl sulfoxide by painting and immersion techniques. Partial control was obtained by using a very low concentration (5%) on one side when involvement was symmetrical. Duration of treatment ranged from 3 to 15 months. Topical dimethyl sulfoxide did not improve the skin induration, range of motion, or Raynaud's phenomenon in the scleroderma patients. No substantial beneficial effect was noted on the healing of ischemic ulcers, and the continuous application of dimethyl sulfoxide did not prevent new ulceratins from developing. Relief of pain was noted in ten of 16 patients, probably due to the local analgesic effect of dimethyl sulfoxide.

Adult↗
PubMed1990

[Tigazon in the therapy of patients with circumscribed scleroderma].

Therapeutic efficacy of tigason in local scleroderma has been demonstrated in 50 patients. The drug has proved effective at all active stages of the process. Its action is favorable in sclerosis and in deep infiltration foci, when traditional therapy, including cuprenil, are of poor efficacy. Tigason altered the course of the disease, making it more benign. The authors recommend this drug, particularly for patients with intolerance of or resistance to the traditional therapy.

Adult↗
PubMed1989

[The immune status of scleroderma patients undergoing electroacupuncture].

Examinations of the immune status of 53 patients with local scleroderma (LS) have revealed changes in the cell-mediated (a deficit of T-lymphocyte population in the presence of an unchanged B-lymphocyte count) and humoral immunity (hyperproduction of the circulating immune complexes, of immunoglobulins A and M, the presence of antibodies to fixed lymphocytes). The patients have been treated by electroacupuncture according to an original method, effecting the points that stimulate suppressed immunity. 1-3 courses of treatment have yielded good results in 83% of the patients, mostly with the early stages of the disease. The treatment has been associated with a positive time course of a number of disturbed parameters of immune homeostasis; a correlation between the clinical and immunological efficacy of therapy has been noted.

Acupuncture Therapy↗
PubMed1998

Classification and epidemiology of scleroderma.

Scleroderma is classified as two separate but related entities, a localized form and a systemic form. The classification scheme for morphea presented here is that of Peterson et al, which divides morphea into five categories: plaque, generalized, bullous, linear, and deep. Using this system, these authors estimated the incidence rate of localized scleroderma to be 27 new cases per million population per year. Overall survival was similar to that of the general population. There was a preponderance of female cases (approximately 3:1) for all forms of morphea except for linear scleroderma, which had an even sex distribution. Systemic scleroderma is divided into limited and diffuse disease based on the extent of skin involvement. Recent estimates have placed the incidence rate of systemic sclerosis in the United States at 19 new cases per million adults per year, with an overall prevalence of 240/million adults. The female-to-male ratio is approximately 5:1. The prevalence of scleroderma varies by geographic region and ethnic background and is higher in the United States than in Europe or Japan. Although systemic sclerosis survival has improved over the past two decades, with 5-year survival over 80%, long-term survival is significantly lower than expected, and morbidity is considerable.

Adult↗
PubMed1986

[Serum lysosomal hydrolases in various forms of scleroderma].

The activities of several lysosomal hydrolases (beta-galactosidase, beta-hexosaminidase, alpha-mannosidase, beta-glucuronidase and acid phosphatase) were determined in serum and blood lymphocytes from patients affected with scleroderma. Statistical comparisons between means of patient and control groups (Student's t test) showed a significant difference in serum beta-galactosidase and acid phosphatase between patients and controls (serum beta-galactosidase: 36.5 +/- 22 nmol/h/ml in the scleroderma group versus 24 +/- 13 nmol/h/ml in the control group; serum acid phosphatase 853 +/- 345 nmol/h/ml in the scleroderma group versus 634 +/- 295 nmol/h/ml in the control group). These differences were significant (p less than 0.01). Both enzyme activities were also significantly increased in the group with systemic scleroderma, but the difference was less in the group with localized scleroderma (this is discussed in terms of statistics and pathophysiology). The other enzyme activities determined were not significantly modified. The validity of these results is discussed, together with their diagnostic value and with the pathophysiological hypotheses put forward to explain the high levels found.

Adult↗
PubMed1998

Topical calcipotriene for morphea/linear scleroderma.

BACKGROUND: Morphea and linear scleroderma are characterized by erythema, induration, telangiectasia, and dyspigmentation. There is no universally effective treatment. Oral calcitriol has been beneficial in the treatment of localized and extensive morphea/scleroderma, but the use of topical calcipotriene has not been reported. OBJECTIVE: The purpose of this study was to evaluate the efficacy and safety of topical calcipotriene 0.005% ointment in the treatment of localized scleroderma. METHODS: In a 3-month open-label study, 12 patients aged 12 to 38 years with biopsy-documented active morphea or linear scleroderma applied calcipotriene ointment under occlusion twice daily to plaques for 3 months. The condition of each patient had previously failed to respond to potent topical corticosteroids and, for some patients, systemic medications. Efficacy was assessed at baseline, 1 month, and 3 months. Levels of serum ionized calcium, intact parathyroid hormone, and 1,25-dihydroxyvitamin D and of random urinary calcium excretion were measured. RESULTS: During the 3-month trial, the condition of all 12 patients showed statistically significant improvement in all studied features. No adverse effects were reported or detected through laboratory monitoring of mineral metabolism. CONCLUSION: Topical calcipotriene 0.005% ointment may be an effective treatment for localized scleroderma, but double-blind placebo controlled studies are needed for confirmation.

Administration, Topical↗
PubMed1977

Ultrastructure of cutaneous cellular infiltrates in scleroderma.

Electron microscopy of the skin was performed in ten patients with systemic and four with localized scleroderma. The following three groups of cells were identified: (1) mature lymphocytes, T lymphoblasts, immature plasma cells, and plasma cells; (2) fibroblasts, fibrocytes, and fibroblast-like cells; and (3) macrophages, undifferentiated mesenchymal cells, and monocytes. Inthose specimens with mononuclear cell infiltrates, the most common cells were lymphocytic-types, macrophages, and fibroblasts with well developed rough endoplasmic reticulum. In the specimens at the fibrotic stage, fibroblasts and histiocytic-type cells predominated. This study suggests that cellular and humoral immunity may play a role in the pathophysiology of scleroderma.

Adult↗
PubMed1989

Histochemical differentiation of localized morphea-scleroderma and lichen sclerosus et atrophicus.

Dermatologic literature has debated the occurrence of concomitant morphea-scleroderma (M-S) and lichen sclerosus et atrophicus (LSA) for sometime. Presentation of a case which has the appearance of both M-S and LSA creates a diagnostic dilemma frequently unresolved even by histopathology. Routine hematoxylin and eosin stained sections may add to the confusion and the difficulty of the differentiation, but examination for the presence or absence of elastic fibers in the upper corium of the lesions affords a definitive separation of these two conditions.

Adolescent↗
PubMed1991

[Type IV collagen and laminin levels in the sera from patients with systemic scleroderma (PSS)].

Serum type IV collagen 7S and laminin P1 levels were measured with radioimmunoassay in 33 patients with systemic scleroderma (PSS), 6 localized scleroderma (LS), and one mixed connective tissue disease (MCTD). Serum type IV collagen 7S levels were higher in PSS (5.11 +/- 1.11 ng/ml:m +/- SD) and LS (4.68 +/- 0.46 ng/ml) than in normal controls (3.90 +/- 0.85 ng/ml) (p less than 0.001). Serum laminin P1 levels were also significantly higher in PSS (1.75 +/- 0.34 U/ml) and LS (1.38 +/- 0.20 U/ml) compared to the controls (1.19 +/- 0.16 U/ml) (p less than 0.001 and p less than 0.01, respectively). A significant correlation between these two values in PSS was found (r = 0.465, p less than 0.02). These results suggest that the measurements of these values may serve as a marker of PSS.

Adult↗
PubMed1998

Scleroderma-like disorders.

Scleroderma-like disorders are widely disparate conditions mimicking either systemic sclerosis or cutaneous localized scleroderma, not infrequently displaying features of both. Some are exclusively sclerotic, some scleroatrophic with prevailing sclerosis or atrophies. The recognition of scleroderma-like disorders is of practical importance because by establishing the cause of the disease, it is possible to introduce an effective therapy, as in scleredema Buschke or scleredema diabeticorum, sclerodermiform porphyria, Borrelia burgdorferi-induced sclerodermiform acrodermatitis atrophicans, sclerodermiform phenylketonuria, drug-induced conditions, and so on. Scleroderma-like disorders strongly suggest that the pathogenesis of skin sclerosis and internal involvement may be divergent, and of various causes. Some of them, such as atrophoderma Pasini-Pierini or progressive facial hemiatrophy, frequently overlapping with scleroderma, make the differentiation very difficult, if at all possible, and the diagnosis is often arbitrary. Some, as sclerodermiform graft-versus-host reaction, point to the autoimmune origin of scleroderma. The amply-covered congenital sclerodermiform conditions present a large spectrum of still not widely known and extremely heterogeneous syndromes, associated with numerous anomalies and/or malignancies.

Adolescent↗
PubMed2002

[Presence of the antinuclear antibodies and antibodies to Borrelia burgdorferi among patients with morphea en plaque, deep linear scleroderma and atrophoderma Pasini-Pierini].

Circumscribed scleroderma belongs to the groups of the diseases characterized by fibrosis of the skin and the deeper tissue. According to the published data, 47% to 76% patients with circumscribed scleroderma present antinuclear antibodies (ANA) as well as antibodies to Borrelia burgdorferi which is believed to be causative factor of the disease. The study was conducted in the group of 50 consecutive patients with circumscribed sclerosis (en plaque, deep linear, atrophoderma Pasini-Pierini). Antinuclear antibodies were detected in 18% of the patients in the titer of 1:40 to 1:320. There was statistically significant correlation between activity of the disease and extensiveness of the skin lesions as well as location on the lower extremities although there was no correlation to the clinical symptoms of the disease. In 28.5% of the patients we found antibodies to Borrelia burgdorferi with any correlation to the type of localized scleroderma or its activity. The role of the ANA with no distinctive specificity and antibodies to Borrelia burgdorferi in the localized scleroderma is still unclear.

Adolescent↗
PubMed1977

[3H]Thymidine labeling of dermal endothelial cells in scleroderma.

The purpose of this study was to estimate [3H] thymidine labeling of endothelial cells of skin capillaries in localized scleroderma (LS) and systemic scleroderma (SS). Skin specimens from 14 patients with SS, 5 with LS, and 9 matched controls were studied by in vitro autoradiography. Capillaries from patients with SS showed a statistically significant increase in endothelial cell labeling when compared to vessels from controls (p less than 0.0005).

Adult↗