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PubMedDate not supplied

[Scleroderma in children].

Fifteen children, ten with localized forms of scleroderma and five with progressive systemic sclerosis (PSS), were reviewed. Age of onset was 2 to 11 years. Mean follow-up was 3.4 years. Localized forms were: "en coup de sabre" in two cases, lineal scleroderma of extremities in five, morphoea in one, and combined lesions in two patients. None of them had systemic symptoms, although two had transient arthritis. Affected skin became atrophic in three cases, "En coup de sabre" lesions were removed surgically in two cases, and there were not recurrences. The other patients continued the same or worse. All five patients with PSS showed extracutaneous symptoms. Three of them with acrosclerosis, Raynaud's phenomenon and dysphagia. One patient died because of pulmonary fibrosis in less than a year. Disease remained unchanged in the other patients. The course of disease does not seems to be affected by treatment with several drugs (asiaticoside, colchicine, diphenyldantoin).

Adolescent↗
PubMed1990

Smooth muscle differentiation in scleroderma fibroblastic cells.

Using antibodies to alpha-smooth muscle actin and desmin on paraffin-embedded formalin-fixed tissue sections, the authors demonstrate that fibroblastic cells of localized and systemic scleroderma lesions express features of smooth muscle differentiation. Eleven of eleven skin specimens of systemic sclerosis patients and two of four skin specimens of localized scleroderma displayed the presence of fibroblasts expressing alpha-smooth muscle actin, a cell population that predominated in areas of prominent collagen deposition. A similar fibroblastic phenotype was found in the esophagus, the liver, and the lung specimens obtained from four patients who died of progressive systemic sclerosis. Immunostaining for desmin, performed on adjacent tissue sections, demonstrated that a minority of these fibroblastic cells present in skin and visceral lesions contained this protein. The authors' observations indicate that scleroderma fibroblasts are phenotypically related to the stromal cells previously identified in hypertrophic scars, fibromatoses, and desmoplasia; they might provide novel criteria for the characterization of scleroderma lesions and help to identify the factors responsible for phenotypic modulations in fibroblastic cells.

Cell Differentiation↗
PubMed1992

Clinical aspects of localized and systemic sclerosis.

Now that renal disease is no longer the leading cause of death in patients with systemic sclerosis, attention has shifted to cardiopulmonary involvement. Several reports addressed the issue of whether the pulmonary vasculature in patients with systemic sclerosis is vasoactive or fixed, and one report addressed the use of high-resolution computed tomographic lung scan to diagnose active alveolitis. The issue of whether the clinical syndrome of thrombotic thrombocytopenic purpura in systemic sclerosis is part of, or is distinct from, scleroderma renal crisis has again been raised. Worsening of cutaneous and visceral systemic sclerosis following radiotherapy was noted in several reports.

Gastrointestinal Diseases↗
PubMed1980

[Respiratory function profile in scleroderma. 20 cases (author's transl)].

This study involved 20 patients, 13 female and 7 male, with a mean age of 49 years, suffering from localized (6 cases) scleroderma or generalized without Raynaud's syndrome (6 cas), with Raynaud's syndrome (6 cas) or acrosclerosis (2 cases). Respiratory function tests included study of volumes, flow rates, CO transfer (TLCO) and blood gases. There were 38% normal chest X-rays, 58% ventilatory disturbances (predominance of restrictive syndrome and decrease in (TLCO) was found in only 25% of cases. The authors emphasize the extent of pulmonary vascular lesions in these patients.

Adult↗
PubMed2005

Systemic sclerosis with unusual panniculitis and overlying discoid lupus erythematosus-like lesions.

A 33-year-old men presented with systemic sclerosis accompanied by both unusual panniculitis and overlying discoid lupus erythematosus (DLE)-like skin changes on the left buttock. The lesion did not completely match either lupus erythematosus profundus or morphea profunda but featured clinical and pathological findings halfway between the two entities. No case report of co-existence of systemic sclerosis and morphea profunda was found in the literature, but there are three case reports of patients with systemic sclerosis and lupus erythematosus profundus. One of the three cases had DLE-like changes on the surface of the lesion, a clinical picture similar to our case. We speculated that the DLE-like changes in the overlying skin were due to local tissue ischemia. The accompanying panniculitis in our case was considered to be unique and somehow different from morphea profunda or lupus erythematosus profundus.

Adult↗
PubMed2000

Gynaecologic history in systemic sclerosis.

The aim of the study was to analyse the gynaecologic history of 150 Brazilian patients with systemic sclerosis (SSc) by comparing the outcome of the pregnancies before and after disease onset and in the two clinical variants of SSc, as well as to assess the effects of the pregnancy on the progress of the disease. A retrospective analysis was carried out of 150 female SSc patients, more than 18 years old, who attended the outpatient clinic of the Unit of Rheumatology of the State University of Campinas. The patients were questioned about the number of pregnancies, deliveries (full-term infants, premature births and twins) and fetal deaths (spontaneous abortions and perinatal deaths). These data were subdivided into pregnancies before and after SSc onset. In those gestations started after disease onset the patients were questioned about the evolution of SSc during the pregnancy. The patients were also asked about dyspareunia and the age at menopause. Thirty-two patients (21 %) had never been pregnant, and only five of them were considered infertile. One hundred and eighteen patients (79%) had a total of 406 pregnancies, with an average of 3.4 per patient; there were 364 pregnancies before and 42 after SSc onset. There were 58 fetal deaths (14% of the pregnancies), 50 of these occurring before and eight after disease onset; 55 were spontaneous abortions and the other three were perinatal deaths. The fertility rate was higher in the limited SSc (3.6) than in the diffuse SSc patients (3.1), although the percentage of fetal deaths and the evolution of SSc during the pregnancy were similar in the two clinical variants. In the pregnancies that occurred after the onset of SSc, the clinical course remained stable in 72% of the cases, worsened in 14% and improved in 14%. Dyspareunia was mentioned by 49 patients (37% of those with an active sexual life). Menopause was reported by 72 patients, predominantly with limited SSc (61 patients). The fertility rate in the postmenopausal SSc patients was 3.9, similar to that observed in general postmenopausal population in Brazil. The analysis of the gynaecologic history in this series of SSc patients showed no increased risk in infertility or spontaneous abortions. The fertility rate in the two SSc clinical variants was higher than that observed in the local global population. Most of the patients who became pregnant after the onset of SSc showed no signs of worsening during the course of the disease.

Adult↗
PubMed2002

[Anti-neutrophil cytoplasmic autoantibody-associated rapid progressive glomerulonephritis complicated with both limited and diffuse scleroderma].

We report two patients with scleroderma, 73-year-old female and 67-year-old female, who developed anti neutrophil cytoplasmic autoantibody (ANCA) associated rapid progressive glomerulonephritis (RPGN). Both patients have had a long history of scleroderma (23 and 14 years, respectively) when ANCA-associated glomerulonephritis occurred. In the first patient, scleroderma was localized in both fingers. She has been followed-up as CREST syndrome rather than systemic sclerosis. The complaints on admission were leg edema and left chest pain in the first patient, and a pyrexia and dyspnea in the second patient. Both patients showed pulmonary manifestation (pleural effusion in the first patient, interstitial pneumonia and alveolar hemorrhage in the second patient, respectively) and rapid progressive glomerulonephritis. Both patients died in spite of corticosteroid therapy. Autopsy findings in the second patient demonstrated crescentic glomerulonephritis and alveolar hemorrhage. Our cases demonstrated that MPO-ANCA associated glomerulonephritis could be associated with limited scleroderma as well as systemic scleroderma. In these condition, the prognosis will be poor if scleroderma seemed to be stable.

Aged↗
PubMed1994

Immunocytochemical localization and serologic detection of transforming growth factor beta 1. Association with type I procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and Raynaud's phenomenon.

OBJECTIVE: To determine the presence of transforming growth factor beta 1 (TGF beta 1) and inflammatory cell markers (HLA-DR and Factor XIIIa) and to compare these with the presence of type I procollagen, in clinically uninvolved and involved skin from patients with different subsets of systemic sclerosis (SSc), and to analyze circulating levels of TGF beta 1 in SSc patients. METHODS: TGF beta 1, HLA-DR, Factor XIIIa, and type I procollagen were detected in skin biopsy sections using a biotin-streptavidin-peroxidase system. Levels of circulating TGF beta 1 were measured using a capture enzyme-linked immunosorbent assay technique. RESULTS: Patients with active diffuse cutaneous SSc (dcSSc) showed minimal TGF beta 1 but significant type I procollagen staining in involved skin, while the clinically uninvolved skin of these patients showed moderate extracellular and intra-epidermal TGF beta 1 immunoreactivity. Patients with limited cutaneous SSc (lcSSc) showed elevated TGF beta 1 staining in both involved and uninvolved skin, as well as procollagen staining. Significant TGF beta 1 reactivity, HLA-DR and Factor XIIIa immunoreactivity, numerous inflammatory cells, and procollagen staining were seen in specimens from patients with morphea. Sequential biopsies suggested the presence of cytokine activity at the earliest stages of disease, which was not maintained with progression of sclerosis. Among the disease groups studied, elevated levels of circulating TGF beta 1 were seen only in patients with morphea. CONCLUSION: The pattern of TGF beta 1 staining in dermal sections from patients with dcSSc, lcSSc, and morphea suggests that this cytokine is important in the pathogenesis of scleroderma. Furthermore, the presence of TGF beta 1 prior to the onset of fibrosis indicates an early involvement of this growth factor, possibly in the inflammatory stage of the disease.

Biomarkers↗
PubMed2002

Sclerodermatous graft-vs-host disease: clinical and pathological study of 17 patients.

OBJECTIVE: To collect and review all cases of sclerodermatous chronic graft-vs-host disease from January 1, 1982, through December 31, 2000. SETTING: University hospital in Madrid, Spain. PATIENTS: During the study period, 493 allogenic bone marrow transplantations were performed. Sclerotic lesions developed in 17 patients. RESULTS: Sclerotic lesions appeared after a mean of 529 days. Previously, 10 (59%) of 17 patients showed a leopard-skin eruption. Sclerosis was generalized in 12 patients and localized in 5. Nine patients presented with rippling of the skin and 8 with lichen sclerosus lesions. We found no anti-Scl-70 or anti-centromere antibodies. Results of histological analysis showed pandermal or deep-dermal sclerosis, slight vacuolar degeneration of the basal cell layer, and follicular damage with follicular plugs. In 6 (50%) of the 12 patients with evaluable biopsy specimens, septal panniculitis was found. Squamous syringometaplasia and mucin deposits were also detected. Treatment with high doses of prednisone and azathioprine helped in 8 of 9 patients. In 12 patients, sclerosis disappeared after 487 days. CONCLUSIONS: Leopard-skin eruption, follicular involvement, ripply skin, and lichen sclerosus lesions have been described poorly or not at all in sclerodermatous graft-vs-host disease. The presence of lichen sclerosus, morphea, septal fibrosis, and fasciitis suggests that the sclerosis can start at and affect any level of the skin. Treatment with prednisone and azathioprine seems to halt the process. Most patients have a good prognosis with treatment. Although most lesions disappear, small areas of fibrosis may remain that do not produce any physical or functional impairment.

Adolescent↗
PubMed1999

Localized morphea in children.

We are following 60 patients with morphea and/or linear scleroderma at the Hospital for Sick Children, Toronto. Lesions may vary in depth from epidermal changes resembling lichen sclerosis, to superficial and deep dermal changes. The subcutaneous tissue, bone and muscle may be involved. There is no good clinical marker of the disease. Because of the variation in the time to cure, it is difficult to evaluate treatment regimens. We are currently using methotrexate weekly either orally or subcutaneously and monthly pulsed steroids for three months.

Child↗
PubMed1998

Pediatric scleroderma.

Scleroderma is a diverse group of conditions which have in common fibrosis of skin and other tissues. Although less common in children than in adults, these conditions are an important cause of morbidity and occasional mortality when they occur in the pediatric population. Children are more likely than adults to develop localized forms of scleroderma, and because of the impact on growth, these can result in major facial or limb asymmetry, flexion contractures, and disability. Management approaches must take into consideration the effect of medications on the child (for example, growth failure and osteoporosis from corticosteroids) as well as the psychosocial impact of chronic illness and physical deformity on the child and family. This article describes the types of scleroderma identified in children, reviews epidemiologic and etiologic factors, and discusses management options. Because this is a rare group of diseases managed by both dermatologists and rheumatologists, large series of patients are rare, and controlled studies of management are not available.

Adolescent↗
PubMed1983

Nailfold capillary abnormalities in childhood rheumatic diseases.

The nailfold capillary patterns of 84 patients with a variety of childhood rheumatic diseases and 34 normal control subjects were observed. Distinctive morphologic abnormalities with capillary dilation and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis and with scleroderma (P less than 0.001). Among those with scleroderma, capillary abnormalities were found in all nine patients with systemic disease and in none of 10 patients with cutaneous disease only (Fisher's exact P less than 0.001). Of 25 patients with dermatomyositis for whom muscle biopsies were available for analysis, abnormal nailfold capillary pattern was found with highest prevalence in patients with two or more specific vascular lesions noted on biopsy (Fisher's exact P = 0.041). Nailfold capillary abnormalities are present in distinct populations of childhood rheumatic diseases, reflect the underlying vasculopathy of childhood dermatomyositis, and may be of diagnostic value in distinguishing localized from systemic scleroderma.

Adolescent↗
PubMed1995

Scleroderma and occupational exposure.

OBJECTIVES: A case-referent study was conducted in the province of Trento, Italy, to investigate the possible association between occupational exposure and scleroderma (systemic and localized variants). METHODS: Twenty-one cases of scleroderma were ascertained from the computerized admission files of all of the local hospitals from 1976 to 1991. For each case, two age- and gender-matched referents were selected that were discharged from the hospital on the same day as the case. The subjects were interviewed with a structured questionnaire which included items for personal, work, and medical history. Exposure to organic solvents, other selected chemicals, silica dust, and hand-arm vibration was carefully investigated. RESULTS: A significant association was found between exposure to organic solvents (aromatic hydrocarbons) and scleroderma [odds ratio (OR) 9.28, 95% confidence interval (95% CI) 1.08-243.8]. For the men, the OR for exposure to silica was 5.20 (95% CI 0.48-74.1), whereas for the women it was 2.11 (95% CI 0.20-22.0) for exposure to other selected chemicals. CONCLUSIONS: The results of this case-referent study are consistent with those of previous case reports on the important role of occupation in the etiology of scleroderma. In particular, our findings confirm that exposure to organic solvents and silica is associated with this rare connective tissue disease.

Case-Control Studies↗