Cases reported "Scleroderma, Systemic"

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1/14. Treatment of scleroderma skin ulcers using becaplermin gel and hydrocolloid membrane.

    skin ulcers can be very painful and detrimental in patients with systemic sclerosis, or systemic scleroderma. A brief review of scleroderma skin ulcers is presented, as well as a case study that demonstrates the effectiveness of becaplermin gel supplemented by oral immunosuppressive agents in the treatment of ulcers resulting from systemic sclerosis. The time to healing (approximately 3 months) was comparable to that associated with the oral agents and surgical interventions specifically designed to help heal scleroderma ulcers. Except for incisional biopsy, no surgical procedures were performed.
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2/14. Nocardial endophthalmitis: report of two cases studied histopathologically.

    We report two cases of nocardial endophthalmitis. Case 1 is a 66-year-old man whose left eye was enucleated about one month after onset of decreased vision. Ophthalmoscopic examination disclosed multiple choroidal masses. Six weeks before the onset of ocular manifestations he had undergone a prolonged carotid endarterectomy with intraoperative complications. The source of the ocular infection was probably exogenous. Case 2 is a 49-year-old woman who had systemic sclerosis (scleroderma) with severe pulmonary insufficiency. She had received moderate doses of corticosteroids. Seventeen months after initiation of therapy she developed ocular manifestations leading to enucleation of the eye one month later. Histopathologically, the enucleated eyes in both cases showed numerous branching, Gram-positive, filamentous organisms involving mainly the plane of Bruch's membrane and the subretinal space. The nosological and microbiological aspects of nocardiosis are reviewed.
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3/14. carpal tunnel syndrome as the initial manifestation of scleroderma.

    Two cases of scleroderma (systemic sclerosis) in which carpal tunnel syndrome was the initial manifestation are described. The development of persistent bilateral swelling of the hand after division of the transverse carpal ligament was a clue to the underlying disorder.
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keywords = ligament
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4/14. Symmetrical widening of the periodontal ligament space in a patient with multiple systemic problems.

    Progressive systemic sclerosis is an uncommon disease that affects the collagen in multiple organ systems. Characteristic oral manifestations that may complicate dental treatment include limited opening, TMJ dysfunction, mucogingival problems, xerostomia, and pathological resorption of the mandible.
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5/14. Bullous sclerodermalike changes in chronic graft-vs-host disease.

    Cutaneous sclerodermalike changes are a well-documented manifestation of chronic cutaneous graft-vs-host reaction. We describe a patient with chronic cutaneous graft-vs-host reaction who developed vesicles and bullae on sclerodermoid skin 18 months after bone marrow transplantation. The vesicles and bullae were subepidermal in location by light microscopy and were associated with dilated lymphatics and a sparse perivascular mononuclear cell infiltrate. No deposition of immunoreactants was seen by immunofluorescent microscopy. Electron microscopy confirmed the presence of a subepidermal blister beneath an intact basement membrane zone and surrounded by marked dermal edema. We postulate that localized lymphedema may play a role in the development of these vesicles and bullae.
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6/14. Linoleate and possibly linolenate deficiency in a patient on long-term intravenous nutrition at home.

    A 39-year-old female with scleroderma was maintained on total parenteral nutrition (TPN) at home for four years. She received 2 units of a 10% fat emulsion per week in which 55% of the fatty acids were from linoleate and 7% from linolenate. She was initially placed on TPN because she had difficulty in swallowing due to scleroderma. At the end of four years she had a triene:tetraene ratio of greater than 1. There was evidence of alterations in membrane function due to essential fatty acid deficiency including CNS involvement (blindness, impaired hearing and disorientation) as well as respiratory insufficiency. The diagnosis of membrane involvement was made from gas chromatography (gc) and gc-mass spectroscopy (ms) analysis of red cell membranes which were deficient in linoleate. The patient's immediate cause of death was from respiratory insufficiency.
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7/14. Clinical effectiveness of apheresis in the treatment of progressive systemic sclerosis.

    Scleroderma is a chronic disease of unknown origin characterized by diffuse sclerosis of the connective tissue of the integument and other organs. As in systemic lupus erythematosus (SLE), rheumatoid arthritis and dermatomyositis, a variety of antibodies may be found in the serum, suggesting that immune mechanisms may be involved. In two uremic patients with scleroderma, the effects were studied of the removal, by pheresis, of antinuclear antibodies, immune complexes, and immunoglobulin factors perpetuating the inflammatory response. We used a membrane plasmapheresis with a hollow-fiber type plasmafilter (Plasmaflo, Asahi) connected online with a hemofilter for conventional dialysis. The aim of this preliminary study was to induce a remission of the edematous inflammatory phase. The correlation of a improvement in clinical response and a decrease in the levels of immune complexes or other factors of inflammation was observed. In our view membrane-plasmapheresis is a process safely carried out by the hemodialysis staff and it may be of clinical effectiveness, especially, in patients with life-threatening complications of progressive systemic sclerosis.
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8/14. Peripheral neuropathy in the course of progressive systemic sclerosis: light and ultrastructural study.

    We present the case of a woman with progressive systemic sclerosis (PSS) in whom the usual symptoms were preceded by a rapidly progressive peripheral neuropathy. Few cases of peripheral nerve involvement have been described. For the first time we report an ultrastructural study of an affected peripheral nerve and muscle. In the sural nerve we found an almost complete loss of myelinated fibers. schwann cells showed an abnormal hyperplasia of their basal membranes and structural signs of denervation. Spindle-shaped banded structures were seen in the cytoplasm of schwann cells and in the endoneurium. On the basis of these ultrastructural data some hypotheses on the pathogenetic mechanism of this neuropathy are discussed.
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9/14. Bronchioloalveolar carcinoma in progressive systemic sclerosis. Report of a case diagnosed by fine needle aspiration cytology.

    BACKGROUND: Progressive systemic sclerosis (PSS) is a systemic disorder involving many internal organs, including the lungs. The occurrence of a pulmonary tumor has occasionally been reported, especially in long-standing cases. Bronchioloalveolar carcinoma is the most prevalent histologic type. CASE: A 35-year-old male with a 12-year history of PSS presented with exertional dyspnea and pain in the left side of the chest. Fine needle aspiration cytology (FNAC) showed abundant cellular material with large, monolayered sheets and papillary structures. The neoplastic cells had uniform round to oval nuclei and spacious cytoplasm. Some had intranuclear vacuoles. phosphotungstic acid-hematoxylin (PTAH) stain revealed structures in the form of prominent cell membranes rimming the free surfaces of the tumor cells. The overall cytologic picture was interpreted as consistent with bronchioloalveolar carcinoma. The patient's condition did not permit surgery. CONCLUSION: In this case the development of bronchioloalveolar carcinoma occurred over a 12-year period following the diagnosis of PSS. This case emphasizes diagnosis of the tumor solely by FNAC. PTAH stain was also helpful in the cytologic diagnosis.
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10/14. Progressive systemic sclerosis in a child: case report.

    A case of pediatric progressive systemic sclerosis is reported and a literature review concerning medical and dental aspects of this condition is provided. Systemic features include sclerodactyly, Raynaud's phenomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, esophageal hypomotility, pulmonary fibrosis and heart failure. oral manifestations include reduced interincisal distance, xerostomia, telangiectasia, increased periodontal ligament width, osseous resorption of the mandible, periodontal disease, and increased decayed, missing, and filled teeth (DMFT). The prognosis is difficult to predict because spontaneous remission has been documented, but death may result from extensive visceral involvement (heart, kidney, and lung).
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keywords = ligament
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