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PubMed1984

[Digestive localizations of scleroderma].

Systemic scleroderma involves the gastro-intestinal tract in over 50 p. 100 of cases, the commonest target organs being the oesophagus, the small intestine, the colon and the stomach in that order. The G-I symptoms of this collagenosis are all related to disorder of motility secondary to disturbances of innervation and then to atrophy of the smooth muscle and fibrous infiltration. Oesophageal involvement results in gastro-oesophageal reflux and/or dysphagia due to the lack of tonicity of the lower oesophageal sphincter and a reduction of peristalsis. Disease of the small intestine may cause pseudo-intestinal obstruction or a secondary malabsorption syndrome due to abnormal intraluminal bacterial flora. Colonic involvement causes severe constipation with formation of faecoliths. Finally, scleroderma may be complicated by an acute abdominal syndrome: occlusion due to diffuse reduction in small intestinal motility, peritonitis due to perforation of the small intestine, ileo-colonic infarction, gastro-intestinal haemorrhage complicating telangiectasia. Treatment is purely symptomatic: classical remedies for gastro-oesophageal reflux and its complications, and antibiotics for malabsorption syndromes.

Anus Diseases↗
PubMed2001

Disseminated scleroderma of a Japanese patient successfully treated with bath PUVA photochemotherapy.

BACKGROUND: Localized scleroderma is known to be resistant to therapies. Recently, it has been reported that bath PUVA photochemotherapy is effective for treating this dermatosis. OBJECTIVES: Although according in earlier reports mainly white populations have been treated successfully with bath PUVA therapy, there is little knowledge of whether it is effective in treating colored populations. We treated a 64-year-old Japanese woman suffering from disseminated scleroderma with bath PUVA photochemotherapy to see its effects. CONCLUSION: Although rather high cumulative UVA doses were required for this patient compared with those needed in earlier reports, no adverse effects were observed. The lesions were markedly improved, suggesting that this therapeutic modality is well-tolerated and useful for colored patients such as the Japanese. Furthermore, it turns out that the thermographical assessment is useful to estimate clinical improvement of this sclerosing skin disorder.

Asian People↗
PubMed1993

[Correlation between various autoantibodies in scleroderma patients and their clinical features].

We studied the antibodies of cell structural localized antibody and the correlation between the new classifications and their clinical features. The results suggest that antikinetochore antibody is the apparent indicator of CREST syndrome, anticentrosome antibody the apparent indicator of local scleroderma with mild clinical features and better prognosis, antinucleous antibody the apparent indicator of diffuse scleroderma with most severe clinical features, high mortality and poor prognosis. New classifications 13 of diagnostic value in clinical grouping and prognosis estimation.

Adolescent↗
PubMed1983

Cyclofenil in childhood scleroderma.

Treatment with cyclofenil for 9 months of a 10-year-old girl with localized scleroderma is reported. No clinical improvement was achieved. The drug was discontinued due to hepatotoxicity.

Child↗
PubMed1997

Reconstruction of scleroderma of the breast.

Although limited to a single case, our study suggests that patients with localized scleroderma of the breast can incur developmental impairment leading to severe breast deformity. The results of any of several methods of breast reconstruction were not available to us for review because of the rarity of this clinical problem. Tissue expansion allowed our patient to avoid the additional scarring and possible functional loss associated with the transfer of autologous tissues. Its successful completion in this one patient indicates that it can be considered for other patients encountered with similar unusual findings.

Adolescent↗
PubMed1991

Focal scleroderma and severe cardiomyopathy. Patient report and brief review.

A 21-month-old infant presented with simultaneous localized scleroderma and severe cardiomyopathy with heart failure. Cardiac abnormalities and serological changes (positive rheumatoid factor assay, elevated IgM and IgG levels, and elevated erythrocyte sedimentation rate) reverted to normal with prednisone therapy, and there was substantial, though incomplete, resolution of her skin changes during the same period. To our knowledge, this is the first patient with definite, clinically significant cardiac involvement associated with focal scleroderma. The possibility of internal organ involvement, including cardiac involvement, must be considered with focal scleroderma as well as with progressive systemic sclerosis.

Cardiomyopathy, Dilated↗
PubMed1999

[Severe course of a mutilating pansclerotic circumscribed scleroderma in childhood. Clinical aspects and therapy].

Disabling pansclerotic morphea of childhood is the most severe variant of localized scleroderma. It is characterized by rapid progression of deep cutaneous fibrosis expanding over large areas of body surface. The prognosis in terms of normal life activity is poor and the disease may even take a fatal course. Presented is a case with extremely severe and rapidly progressive lesions resulting in cutaneous ulcerations joint contractures, and multilaing deformities of the extremities. Histopathological analysis revealed extensive intravascular calcinosis of the small vessels, which may be an important factor in the pathogenesis of this poorly understood disease. UVA1-phototherapy was performed and induced a softening of sclerosis and a distinct decrease of skin thickness. Based on these observations UVA1-phototherapy may be promising in the treatment of extensive sclerotic disease of this kind, and possibly other diseases accompanied by excessive sclerosis.

Adolescent↗
PubMed2002

Linear scleroderma and autoimmune hemolytic anaemia.

Coexistence of localized scleroderma with other autoimmune disorders is not seen frequently. It has been reported to occur with myasthenia gravis, hemiatrophy and systemic lupus erythematosus. In this report we describe an association wherein linear scleroderma coexisted with autoimmune haemolytic anaemia.

Adult↗
PubMed1995

Circulating Fc gamma receptor-specific autoantibodies in localized and systemic scleroderma.

BACKGROUND: Anti-Fc gamma receptor (anti-Fc gamma R) autoantibodies occur in patients with systemic scleroderma. Their clinical significance is unknown. OBJECTIVE: Our purpose was to determine the incidence of anti-Fc gamma R autoantibodies in patients with localized and systemic scleroderma and to examine the relation between these autoantibodies, the severity of the disease, and the presence of other autoantibodies. METHODS: Patients were placed into three clinical groups: three had diffuse systemic scleroderma, 47 had limited systemic scleroderma, and nine had localized systemic scleroderma. Antinuclear antibody titer and pattern were measured by indirect immunofluorescence with human epithelial (HEp)-2 cells and tissue sections, whereas anti-Scl-70 antibodies were measured by gel diffusion technique. Anti-Fc gamma R autoantibodies were measured in serum from patients and from 25 healthy persons by enzyme-linked immunosorbent assay with human recombinant Fc gamma RII (CD32) and Fc gamma RIII (CD16). RESULTS: Anti-Fc gamma R autoantibodies were detected in 54% of patients and in none of the healthy control subjects. Autoantibodies were present in all three clinical groups and were most frequently directed against Fc gamma RIII. Correlation between patients' clinical and laboratory data and anti-Fc gamma R autoantibodies could not be demonstrated. CONCLUSION: The presence of anti-Fc gamma R autoantibodies in the serum of patients with either systemic or localized scleroderma and the lack of these autoantibodies in healthy persons suggest that they may play a role in the pathogenesis of these diseases.

Adolescent↗
PubMed1987

Alterations in scleroderma fibroblast surface glycoproteins associated with increased collagen synthesis.

Fibroblasts were cultured from affected and unaffected skin sites of 6 patients with localized scleroderma. As a parameter of fibroblast activation, collagen synthesis and cellular pro alpha 1(I)collagen mRNA levels were measured. Cell surface glycoproteins were labelled with the periodate/borohydride method and fractionated electrophoretically. A distinct reduction in the relative amount of surface glycoproteins in the 120 kDa region was observed in two affected cell lines producing increased amounts of collagen and in one affected cell line with normal collagen production when compared to unaffected fibroblast lines. Other, non-systematic alterations in the surface glycoproteins of the affected cell lines were also detected. Compared to unaffected and healthy control fibroblasts no alterations in the surface protein profiles were seen in the other three affected cell lines. These cells did not show an increase in collagen production either. The results suggest that the activation of collagen synthesis found in scleroderma fibroblasts might be connected with alterations in the normal cell surface glycoprotein pattern.

Adult↗
PubMed1991

Clinical aspects of localized and systemic scleroderma.

A number of reports of potential etiologic agents of localized and systemic scleroderma appeared in the past year, including alterations in tryptophan metabolism, use of appetite suppressants, and exposure to silicone. An infectious agent, Borrelia burgdorferi, was found not to be implicated in localized scleroderma. The improvement in outcome of systemic scleroderma complicated by renovascular hypertension was highlighted in several papers, as was the emerging importance of cardiac and pulmonary involvement. Recent advances in the early detection and evaluation of cardiac and pulmonary complications of scleroderma are discussed.

Humans↗
PubMedDate not supplied

Progressive hemifacial atrophy with linear scleroderma.

We describe a 4-year-old girl with hemifacial atrophy. She had a linear white-colored sclerotic plaque on the right submandibular area of skin. Histologic findings of the lesion were consistent with a diagnosis of scleroderma. The relationship between progressive facial hemiatrophy and linear scleroderma are discussed. We think that linear scleroderma of childhood and hemifacial atrophy have considerable clinical overlap and these two syndromes appear to be manifestations of the same or related pathogenic processes. Recently, the beneficial effects of 1.25-dihydroxyvitamin D3 (calcitriol) were reported in adults and in children with linear scleroderma. We assessed the efficacy of oral calcitriol treatment in our patient. Our result indicates that calcitriol may be an effective agent for treating localized scleroderma in children.

Calcitriol↗
PubMed1983

[Post-traumatic nodular scleroderma].

Scleroderma of rare appearance in children appears in minor scale as to the five per cent on the whole incidence of this collagen disease. The children usually present localized scleroderma and at times associated with other pathologies, traumatisms and injections were referred. Two patients aged 5 and 13 years old are presented, both with nodular lesions on anterolateral thigh area, and in the right buttock respectively. The patches of a side bigger than the palm of hand were only touchable and the skin that covered them only showed a slight hyperpigmentation in the edge in one of the cases. The limits were not precise and the nodulose surface was irregular. The evolution was as of two and three years, right after inoculation of antitetanical vaccination and puncture thorn of Yuca leaves. The histological control showed intensive phenomenons of fibrohyalinosis covering almost all the dermis. The studies of the laboratories didn't produce interesting data. The histological and clinical set of symptoms shows differences with the esclerodermic like states as a consequence of excipient of vitamin K, B 12, norhydroxprogesterone and anti-tetanic serum, in which they settle in the cellular subcutaneous tissue and they involution spontaneously. There are also differences with the paniculitis artefacta and with the lincar morphea associated to bifid spine. At last the authors make special mention of the case described by Desmons of progressive linear scleroderma right after the triple vaccination. The nodular or subcutaneous scleroderma is a clinical form in which the histological alteration decays in deep dermis and superficial hypodermis. The cases shown suggest a conection between a previous traumatism and the nodular scleroderma.

Adolescent↗
PubMed1988

Interferon-alpha and interferon-gamma reduce excessive collagen synthesis and procollagen mRNA levels of scleroderma fibroblasts in culture.

The effects of interferon-alpha and interferon-gamma on collagen synthesis and mRNA levels of type I and type III procollagens were studied in skin fibroblasts cultured from affected and unaffected skin sites of two patients with localized scleroderma (morphea). Both scleroderma cell lines exhibited elevated type I and type III procollagen mRNA levels to account for the increased procollagen synthesis, when compared to the unaffected controls. Interferon-gamma treatment resulted in a dose-dependent reduction in collagen synthesis and procollagen mRNA levels in scleroderma fibroblasts. A 72-h exposure to interferon-gamma reduced procollagen mRNA levels in the scleroderma fibroblast lines to the levels exhibited by the unaffected control fibroblasts. The suppressive effect of interferon-alpha on procollagen mRNA levels was somewhat weaker than that of interferon-gamma. The results suggest potential use of interferon-gamma in treatment and prevention of human fibrotic conditions.

Cells, Cultured↗
PubMed2003

Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship?

Extracutaneous calcifications are rare in scleroderma and related conditions. We describe a female patient with linear scleroderma en coup de sabre and a longstanding clinical history of tonic and clonic convulsions. Radiographic study showed extensive cerebral calcifications in the right occipital hemisphere, homolateral to the involved side of her face. This report further suggests a relationship between localized scleroderma and neurological manifestations. Brain imaging studies should be routinely performed in scleroderma patients exhibiting neurological manifestations, especially seizure disorder.

Adult↗
PubMed1995

[Association of lichen sclerosus and monomelic scleroderma].

INTRODUCTION: Coexistence of lichen sclerosus and scleroderma is well demonstrated. However, clinical and histological lesions of lichen sclerosus and scleroderma, in a linear pattern, do not seem to have been reported. CASE REPORT: We report the case in a six years old boy, of both lichen sclerosus and linear scleroderma lesion that involved his left lower limb. Immunological and inflammatory investigation was normal or negative, as well as, bone and muscles analyze of the leg. Further outcome was benign. DISCUSSION: This new case confirms the possibility of an association, inside a same limb, of lichen sclerosus and localized scleroderma, and supports the concept of a common etiological process in these two diseases. In an other hand, it could reflect the possibility of two distinct clinical expressions of the same pathogenic process.

Atrophy↗
PubMed1991

Unilateral enophthalmos in systemic scleroderma.

Atrophy of orbital fat is a reported complication of localized scleroderma but not of systemic scleroderma. Here we present a case of systemic scleroderma with unilateral enophthalmos. Orbital fat atrophy was the presumed cause on computerized tomography.

Adipose Tissue↗