Cases reported "crest syndrome"

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1/51. Early assessment of pulmonary involvement in limited scleroderma. A case report.

    Limited scleroderma is typified by insidious progression of skin involvement. The onset of internal organ involvement is delayed until the second decade, the lungs being the most important from the prognostic point of view. Early detection of pulmonary lesions is of paramount importance. This paper presents a 16-year-old male patient with a history of Raynaud's phenomenon followed by progressive tightening of skin over the fingers, hands and face. He had early pulmonary involvement detected by high resolution computed tomography (HRCT) and proven by histopathologic examination as usual interstitial pneumonia; even the chest x-ray and pulmonary function tests were normal. A combination of prednisolone and D-penicillamine was planned for treatment because of his having both pulmonary and gastrointestinal system involvement. 99 m technetium diethylenetriamine pentaacetate (99 m Tc-DTPA) test is very sensitive for pulmonary lesions and it has shown a rapid clearance in the early stage. This method is also useful for following up the therapeutic trial. ( info)

2/51. peritoneal dialysis-associated peritonitis caused by Propionibacteria species.

    There are an increasing number of reports about unusual causes of peritonitis in peritoneal dialysis (PD) patients. The Propionibacteria species is a microorganism that is a normal skin flora. Under the presence of certain risk factors, it may produce serious infections. patients at risk of having Propionibacteria sp infections have malignancy, diabetes mellitus, foreign bodies, or immunodeficiency. We describe a PD-associated peritonitis in a 51-year-old woman that was caused by Propionibacteria sp. This patient's risk factors for developing Propionibacteria sp peritonitis include a history of crest syndrome, malignancy of the breast, and recent catheter surgery. To our knowledge, this is the first case of a PD-associated peritonitis caused by Propionibacteria sp reported in the literature. ( info)

3/51. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.

    We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases. ( info)

4/51. crest syndrome and periodontal surgery: a case report.

    crest syndrome is a slowly progressive form of systemic scleroderma. It is characterized by calcinosis cutis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. There are limited reports of dental treatment for patients with this syndrome, and no reports of periodontal surgical procedures. This paper presents a case report of periodontal surgical treatment in a 38-year-old female patient with crest syndrome, and a discussion of the clinical manifestations of the syndrome as they relate to dental treatment. ( info)

5/51. Phlebosclerosis of the colon with positive anti-centromere antibody.

    A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the crest syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the crest syndrome. ( info)

6/51. A case of crest syndrome and myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis.

    We report the first case of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated glomerulonephritis in a patient with crest syndrome. A 74-year-old Japanese man with crest syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) developed rapidly progressive renal failure without elevation of blood pressure. Renal biopsy revealed glomerular sclerosis and fibrous crescents. The MPO-ANCA titer was elevated to 145 EU/ml. When patients with collagen diseases develop rapidly progressive glomerulonephritis, the possibility of MPO-ANCA-associated glomerulonephritis should be kept in mind. ( info)

7/51. autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.

    OBJECTIVE: To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc). methods: We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms. RESULTS: We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found. CONCLUSION: Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process. ( info)

8/51. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.

    Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed. ( info)

9/51. Pulmonary hypertension with limited cutaneous scleroderma (crest syndrome).

    A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as crest syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension. ( info)

10/51. Granulomatous anterior uveitis in a patient with crest syndrome.

    The crest syndrome is a variant form of progressive systemic sclerosis. Apart from the occurrence of keratoconjunctivitis sicca, other types of ocular involvement associated with this variant are quite rare. We present the case of a 73-year-old woman with the CREST variant of progressive systemic sclerosis who developed unilateral granulomatous anterior uveitis. Systemic and laboratory testing failed to suggest evidence for any other associated systemic disease as a possible cause of the granulomatous uveitis. The inflammation was successfully controlled with topical steroids and mydriatics. While a small number of cases of uveitis have been reported in other variant forms of progressive systemic sclerosis, to date there have been no descriptions of uveitis associated with the crest syndrome. ( info)
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