Localized scleroderma associated with sarcoidosis.
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Anti-Inflammatory Agents↗
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Effectiveness of topical calcitriol for localized scleroderma.
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Administration, Topical↗
[Localized scleroderma: lesion "en coup de sabre". Review of the literature and report of a case].
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Child↗
Oesophageal motility and lower oesophageal sphincter competence in progressive systemic sclerosis and localized scleroderma.
Oesophageal motility and lower oesophageal sphincter (LOS) competence were investigated in 13 patients with progressive systemic sclerosis (PSS) and in 16 patients with localized scleroderma (LS) by means of oesophageal manometry and 24-h pH monitoring of the distal oesophagus. Results were compared with those of a control group consisting of asymptomatic volunteers. Marked abnormalities in oesophageal motility and in acid exposure in the distal oesophagus were observed in PSS patients only. The mean resting pressure of the LOS was 10.1 +/- 1.5 mmHg in PSS, 21.4 +/- 1.1 mmHg in LS, and 23.8 +/- 2.0 mmHg in asymptomatic controls. Overall sphincter length was 24.1 +/- 3.4 mm in PSS, 31.1 +/- 1.6 mm in LS, and 39.0 +/- 2.0 mm in the control group. Spincter abdominal length was 12.1 +/- 2 mm, 15.4 +/- 1 mm, and 25.0 +/- 1 mm, respectively. The amplitude and duration of oesophageal waves were markedly reduced at 5, 10, and 15 cm above the LOS in PSS patients, with only the upper part of their gullet being spared. An abnormal acid exposure in the distal oesophagus was observed in 84.6% of PSS patients, whereas only 18.2% (2 of 11) of pH-tested LS patients had an abnormal 24-h pH test. These data show that a marked oesophageal involvement is present only in the systemic form of scleroderma. Oesophageal tests may be useful for a circumstantial diagnosis whenever the diagnosis of PSS is uncertain; however, their use does not seem to be justified as routine in patients with LS.
Adult↗
Serum levels of tumor necrosis factor and interleukin-13 are elevated in patients with localized scleroderma.
BACKGROUND: The enhanced production of some cytokines may be related to the pathogenesis of localized scleroderma (LSc). OBJECTIVE: To determine whether serum levels of tumor necrosis factor (TNF) and interleukin (IL)-13 are elevated, and whether they correlated with clinical or serological features in patients with LSc. METHODS: Serum levels of TNF and IL-13 were examined by ELISA in 45 patients with LSc and in 20 healthy controls. RESULTS: The frequency of serum TNF detection was significantly higher in patients with LSc (24%) compared with that in healthy controls (0%). The frequency of serum IL-13 detection was also significantly higher in patients with LSc (29%) compared with that in controls (0%). In patients with LSc, elevated TNF levels significantly correlated with the presence of IgM antihistone antibodies, anti-single-stranded DNA antibodies, elevated serum IL-6 levels, the number of linear lesions, and muscle involvement. Elevated IL-13 levels were significantly associated with the number of plaque lesions and the total number of lesions. CONCLUSIONS: These results suggest that TNF and IL-13 may be associated with the development of LSc.
Adolescent↗
Flow cytometry of fibroblasts cultured from skin of patients with localized scleroderma.
Fibroblast cultures were initiated from affected and unaffected skin sites of 6 patients with localized scleroderma. Two of the affected cell lines exhibited more than threefold increases in procollagen production and mRNA levels. All the cell lines were analyzed by flow cytometry to detect a possible heterogeneity of scleroderma fibroblast cultures which could explain the production of excessive amounts of collagen. No evidence of a subpopulation responsible for elevated collagen production was detected using cytoplasmic dot hybridization of cells fractionated by flow cytometry. When compared with the nonaffected controls, all the cell lines from affected skin areas of scleroderma patients were found to exhibit a lower level of cellular autofluorescence, suggesting an alteration in metabolic activity. The results show that the heterogeneity of scleroderma fibroblasts that was found in vivo is lost when the cells are cultured.
Adolescent↗
Identification of fibroblasts responsible for increased collagen production in localized scleroderma by in situ hybridization.
Skin biopsies from seven patients with localized scleroderma (morphea) and from two healthy individuals were studied by in situ hybridization to localize the cells responsible for increased procollagen production. In scleroderma lesions, high levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs were detected in some but not all fibroblasts, suggesting the presence of a subpopulation responsible for the increased collagen production. The levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs in these fibroblasts were clearly elevated compared to control skin specimens hybridized at the same time under identical conditions. Most of the scleroderma samples represented intermediate stages where the fibroblasts containing elevated levels of type I and type III procollagen mRNAs were located in the papillary and upper reticular layer of the dermis. One of the scleroderma samples from an early inflammatory stage of the disease was found to contain activated fibroblasts in all dermal layers and also in aggregates adjacent to inflammatory cell infiltrates. In situ analyses were also performed on cell cultures from affected and unaffected skin of one scleroderma patient. These experiments revealed a homogeneous population of activated fibroblasts in cultures producing high levels of collagen. The results suggest that development of fibrosis in scleroderma could evolve through activation of a certain fibroblast subpopulation. During cell culturing, however, cell selection or uncharacterized regulatory mechanisms appear to modulate the behavior of these cells with respect to collagen production.
Autoradiography↗
Thirteen-megahertz ultrasound probe: its role in diagnosing localized scleroderma.
BACKGROUND: Ultrasound imaging has been shown to be useful for the evaluation of systemic and localized scleroderma (LS). However, its specificity and sensitivity have not been studied. OBJECTIVES: To define morphological ultrasound diagnostic criteria in LS and to test their sensitivity and specificity with a 13-MHz ultrasound probe. METHODS: Forty plaques in 26 consecutive patients with LS were examined and compared blindly with 17 control plaques in 16 patients with skin diseases where LS was in the differential diagnosis. Data were also compared with a normal control group. Five patients were re-evaluated 12-18 months after the first examination. RESULTS: Ultrasound examination disclosed a characteristic dense image resembling a flattened 'yo-yo'. Undulations of the dermis, disorganization, loss of thickness and thickened hyperechoic bands in the hypodermis, and the 'yo-yo' image had a high sensitivity and a high specificity for LS. A 92% sensitivity and a 100% specificity for LS were found when at least four of these five signs were present. CONCLUSIONS: Thirteen-megahertz ultrasound is a valuable tool for diagnosing LS. Morphological ultrasound diagnostic criteria had a high specificity and a high sensitivity.
Adolescent↗
Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.
Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.
Adolescent↗
Localized scleroderma progressing to systemic disease. Case report and review of the literature.
We describe a 15-year-old girl with biopsy-proven morphea who developed progression to systemic disease 2 years after initial presentation. In contrast to other reported patients with localized scleroderma, some of whom have had mild, nonprogressive systemic involvement, this patient developed severe, debilitating disease, with skin tightness, sclerodactyly, esophageal involvement, restrictive pulmonary disease, and myopathy. From the time of her initial evaluation, the patient was positive for antinuclear antibodies (ANA), which were shown to be primarily directed against the Ku antigens. This observation suggests that ANA may be a prognostic indicator for progression to systemic disease.
Antibodies, Antinuclear↗
Serum aminoterminal propeptide of type III procollagen in progressive systemic sclerosis and localized scleroderma.
Sera from 31 patients with progressive systemic sclerosis (PSS), 5 patients with widespread localized scleroderma (LS), and 3 patients with lichen sclerosus et atrophicus were analyzed for aminoterminal propeptide of type III procollagen (PIIINP) using a radioimmunoassay based on human propeptide. Thirty-eight per cent of the patients with PSS had levels above normal range, including all of the 3 patients with diffuse scleroderma. The same applies to 4 of 5 patients with widespread localized LS, while PIIINP in all 3 patients with lichen sclerosus et atrophicus were within normal levels. In patients with acrosclerosis, elevated PIIINP seems to be correlated to rapid progression and extension of lesions. A significant increase in PIIINP was found in a patient following discontinuation of prednisone and cyclophosphamide, while the present investigation did not allow judgement of effects of treatment with either penicillamine or cyclosporin A.
Humans↗
Increased serum levels of soluble vascular cell adhesion molecule 1 and E-selectin in patients with localized scleroderma.
BACKGROUND: The serum levels of soluble forms of vascular cell adhesion molecule 1 (sVCAM-1) and E-selectin (sE-selectin) can be used as a marker of endothelial activation. OBJECTIVE: We investigated whether the serum level of sVCAM-1 and sE-selectin in patients with localized scleroderma (LSc) was correlated with the clinical or serologic features of this disease. METHODS: Serum samples from 59 patients with LSc, 20 patients with systemic sclerosis (SSc) and 29 healthy controls were examined using specific enzyme-linked immunosorbent assays. RESULTS: The serum levels of sVCAM-1 and sE-selectin in patients with LSc were significantly higher than those of the healthy controls. The serum levels of sVCAM-1 and sE-selectin were correlated with both the number of sclerotic lesions and the number of involved areas. CONCLUSION: These results indicate that the serum levels of sVCAM-1 and sE-selectin may reflect the extent of the skin involvement (ie, the disease severity) and may be useful for monitoring the in vivo states of endothelial activation in LSc.
Adult↗
Localized scleroderma or morphea?
Morphea is a frequently mild, benign, and self-limiting skin disease with a less than 1% reported chance of progressing to systemic scleroderma. Morphea is a sufficient and less terrifying name for these disorders than localized scleroderma.
Humans↗
Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid, and other fibrotic skin disorders.
Connective tissue growth factor (CTGF) is a novel peptide that exhibits platelet-derived growth factor-like activities and is produced by skin fibroblasts after activation with transforming growth factor-beta. Coordinate expression of transforming growth factor-beta followed by CTGF during wound repair suggests a cascade process for control of tissue regeneration. We recently reported a significant correlation between CTGF mRNA expression and histologic sclerosis in systemic sclerosis. To confirm the relation between CTGF and skin fibrosis, we investigated CTGF gene expression in tissue expression in tissue sections from patients with localized scleroderma, keloid, other sclerotic skin disorders using nonradioactive in situ hybridization. In localized scleroderma, the fibroblasts with positive signals for CTGF mRNA were scattered throughout the sclerotic lesions with no preferential distribution around the inflammatory cells or perivascular regions, whereas the adjacent nonaffected dermis was negative for CTGF mRNA. In keloid tissue, the fibroblasts positive for CTGF mRNA were diffusely distributed, especially in the peripheral expanding lesions. In scar tissue, however, the fibroblasts in the fibrotic lesions showed partially positive signals for CTGF mRNA. In eosinophilic fasciitis, nodular fasciitis, and Dupuytren's contracture, CTGF mRNA was also expressed partially in the fibroblasts of the fibrotic lesions. Our findings reinforce a correlation between CTGF gene expression and skin sclerosis and support the hypothesis that transforming growth factor-beta plays an important role in the pathogenesis of fibrosis, as it is the only inducer for CTGF identified to date.
Adolescent↗
Localized scleroderma after infection with Epstein-Barr virus.
Over a period of three years (1989-1992) five children suffering from localized scleroderma were seen at the Department of Pediatrics of the University of Trieste. Evidence of a previous infectious mononucleosis (IM) was present in four out of five patients. The clinical history of these four children is reported. The association between the appearance of scleroderma and a previous viral infection is not surprising. However, in the pediatric literature there is only one case of progressive systemic sclerosis (PSS) developing in a 15-month-old girl less than one month after she contracted IM. The presence of shared epitopes between an Epstein-Barr virus protein, BOLF1, and the hypervariable region of HLA associated with the pauciarticular form of JCA, recently reported, could provide a key to the pathogenesis of other collagen diseases such as scleroderma.
Adolescent↗
The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993.
OBJECTIVE: To determine the incidence, prevalence, survival rates, clinical manifestations, and longterm outcome of patients with morphea (localized scleroderma) and its subtypes over a 33 year period in Olmsted County, Minnesota. METHODS: We used the unique data resources of the Rochester Epidemiology Project to review all Olmsted County medical records with any potential diagnosis consistent with morphea (including plaque, generalized, bullous, linear, and deep entities) from 1960 through 1993. RESULTS: We screened 1030 medical records and identified 82 (59 female; 23 male) cases of morphea first diagnosed between 1960 and 1993. All cases were followed until death or migration from Olmsted County, a total of 754 person-years of observation. The annual age and sex adjusted incidence rate per 100,000 population was 2.7 (95% confidence interval 2.1, 3.3). The incidence rate increased significantly over the 33 years (p = 0.0037) on an average of 3.6% per year. The prevalence (estimated using cumulative incidence) at 80 years of age was about 2/1000. 50% of the patients had a cutaneous softening or evidence of disease resolution by 3.8 years' duration. The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 years) compared to 5.5 years in the deep group. Arthralgias, synovitis, uveitis, and joint contractures were more frequent in the linear and deep categories. Although 9 patients (11%) developed some disease related disability over the followup period, this was common (44%) in the deep group. No case of morphea developed severe internal organ involvement and none progressed to systemic sclerosis. The survival rate was not significantly different from the general population (p = 0.409). CONCLUSION: Morphea, and its subtypes, are more common than previously recognized, and can lead to important disability.
Adolescent↗
Localized scleroderma associated with Borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations.
BACKGROUND: Recent reports have implicated Borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). OBJECTIVE: Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. METHODS: Ten patients were examined clinically and by routine microscopy. Biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. RESULTS: Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4+ subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. Patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. CONCLUSION: Our findings suggest that some cases of LS are linked to Borrelia infection.
Adolescent↗
Localized scleroderma following varicella in a three-year-old girl with IgA deficiency.
A three-year-old girl with isolated IgA deficiency developed localized scleroderma immediately after varicella infection. Physiotherapy was started without any drug therapy. Only a minimal clinical improvement was achieved. The connection between IgA deficiency, viral infection and collagen diseases is discussed.
Chickenpox↗