Cases reported "Collagen Diseases"

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1/5. Pneumatosis intestinalis in association with connective tissue disease.

    Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.
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keywords = vessel
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2/5. Case report: fatal gastrointestinal hemorrhage in mixed connective tissue disease.

    We have presented the case of a young woman with MCTD who died of gastrointestinal bleeding due to fibrinoid necrosis of blood vessels of the small and large intestine. This appears to be the first recorded occurrence of such findings in MCTD.
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keywords = vessel
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3/5. aortic rupture in a patient with elastosis perforans serpiginosa (Lutz-Miescher).

    A case of aortic rupture in a 30-year-old women with a rare skin disease, elastosis perforans serpiginosa, is presented. skin lesions are characterized by marked elastic tissue changes. Multiple large arteries and the distal part of the aorta were thinwalled also with aneurysm-like dilatations. Focal fibroelastosis and degenerative changes were present in the vessel walls. The changes in the aorta made the surgical treatment difficult.
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keywords = vessel
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4/5. Childhood-type myositis and linear scleroderma.

    A 5-year-old girl had linear scleroderma on the flexor surface of the right arm; muscle wasting included the shoulder girdle. IgM fluorescence on blood vessels and along dermal-epidermal junction was observed by direct immunofluorescence in biopsied skin. Biceps muscle underlying the plaque of the scleroderma showed atrophy of entire fascicles, perifascicular atrophy, and cellular infiltration around blood vessels that are quite similar to those found in childhood-type dermatomyositis. In addition, various abnormalities, including edema and thickening of basal lamina, were found on blood vessels in muscle tissue. The results suggested that the autoimmune collagen vascular disorder is responsible for this condition.
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keywords = vessel
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5/5. Atypical collagen disease. polymyositis, prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis.

    An autopsy case of polymyositis with prominent hematoxylin body formation in various organs and tissues, florid focal and segmental necrotizing glomerulonephritis, and some manifestations of progressive systemic sclerosis was presented. A 34-year-old woman had erythematous edema on the face, joint pain, muscular weakness, cutaneous sclerosis and Raynaud's phenomenon for about 5 years. She had also a long history of pulmonary tuberculosis. autopsy revealed polymyositis involving skeletal, smooth and heart muscles. This was widespread consisting of focal and extensive primary degeneration of muscle fibers and interstitial inflammatory cell infiltrates near and surrounding small veins and venules accompanied by the derivation of hematoxylin bodies from inflammatory cell nuclei, and in the posterior pharyngeal muscles severely affected, muscle substance was literally erased, leaving interstitial fibrous tissues and blood vessels. This focally restricted, severe necrosis of the skeletal muscle gives a special feature to the polymyositis in the present case. The diagnosis of polymyositis with prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis was applied as a rather descriptive designation, but yet it does not absolutely exclude the possibility of a variant of SLE in an additional effort to search for an underlying disorder.
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