1/34. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/34. Raynaud's phenomenon possibly induced by a compund drug of tegafur and uracil.We describe a 50-year-old woman who noted acral hyperpigmentation, sclerodactyly and Raynaud's phenomenon with 1:160 of antinuclear antibody titer after treatment with a compound drug with tegafur and uracil. Histological findings of the finger and palm included hyperkeratosis, vacuolar degeneration of basal cells, thickened collagen fibers in the dermis, and dilatation of capillary vessels, perivascular mononuclear cell infiltration and melanophages in the upper dermis. IgG, IgA, IgM, C3 and C1q were not deposited in the skin by direct immunofluorescence study. After cessation of the drug, Raynaud's phenomenon and hyperpigmentation disappeared within 1 month and 4 months, respectively, and antinuclear antibody turned negative within 4 months. These observations suggest that tegafur may have caused not only hyperpigmentation in the palms and soles, but also sclerodactyly and Raynaud's phenomenon in the present case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/34. 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.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/34. Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type.Renal disease in systemic sclerosis may present in various patterns. A 66-year-old woman with a history of longstanding limited cutaneous systemic sclerosis of the crest syndrome variant presented with a sudden left foot drop and rapidly progressive renal insufficiency associated with mild proteinuria, a nephritic urine sediment, and a urinary output of 900 mL/d. There was no history of intake of D-penicillamine, and there were no signs of malignant arterial hypertension or microangiopathic hemolytic anemia. Renal histology showed a small vessel vasculitis of the microscopic polyangiitis type. serologic tests showed a marked increase of antineutrophil cytoplasmic antibodies with a perinuclear pattern and an elevated titer of antimyeloperoxidase antibodies. No clinical or laboratory signs of sjogren's syndrome were present. This clinical report adds new information to the spectrum of renal disease in systemic sclerosis. It discusses the association between systemic sclerosis and small vessel vasculitis of the microscopic polyangiitis type as well as the possible meaning of serologic markers.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
5/34. Pseudohypertension in a patient with diffuse scleroderma.Pseudohypertension is the artifactual elevation of blood pressure that occurs secondary to noncompressible blood vessels. It has been described in patients with uremia, diabetes mellitus, and severe atherosclerosis. If unrecognized, the condition may lead to inappropriate and potentially harmful therapy. We report a case of pseudohypertension in a 65-year-old man with diffuse scleroderma. His blood pressure as assessed by conventional sphygmomanometry was at least 240/135 to 145 mm Hg. Intra-arterial blood pressure was found to be 107/52 mm Hg. The severe rise in blood pressure as measured by sphygmomanometry led to the concern of scleroderma renal crisis and potentially harmful therapy. Intra-arterial pressure monitoring confirmed the presence of pseudohypertension, however. This is the first reported case of pseudohypertension in a patient with diffuse scleroderma.- - - - - - - - - - ranking = 4.7370954058064keywords = blood vessel, vessel (Clic here for more details about this article) |
6/34. pityriasis versicolor rubra.We report six typical cases with pityriasis versicolor (PV) rubra, with a background of collagen diseases in five cases and none in one case. Two cases of systemic lupus erythematosus (SLE) and one case of systemic scleroderma (SSc) had both PV rubra and nigra on the trunk. diagnosis of superficial infections of PV was made by microscopic examination of skin scrapings following KOH, and many small whitish colonies were obtained in Sabouraud's slant agar medium culture containing cycloheximide with olive oil in all cases. malassezia sympodialis was isolated from the scales of two different lesions at a 6 week-interval in a same person (a 32 year-old male without SLE nor SSc) by the method of Makimura et al. [5], although the other five cases were not examined for the isolation. Histopathological features of the lesion on the dorsum of the trunk showed no epidermal hyperplasia without elongation of rete ridges and no inflammatory cell infiltration in the dermis, however there was only dilatation of small blood vessels in the dermis, which was reconfirmed capillaroscopically. In the horny layers, several yeastlike and fine filamentous structures were seen which were positive with PAS and Grocott stains. Both clinical and histological features led us to speculate PV rubra. All the patients were treated with anti-fungal ointment, and the lesions diminished in less than 2 weeks. No recurrence has been seen in any of the cases. This PV rubra may be independent from PV nigra, although Horiuchi [2] suggested the earlier lesion occurs in advance of PV alba or nigra.- - - - - - - - - - ranking = 4.7370954058064keywords = blood vessel, vessel (Clic here for more details about this article) |
7/34. Arterial thrombosis in scleroderma.Clinical and pathological observations are presented on five patients with scleroderma who developed thrombosis of a major vessel. Three died following intestinal infarction or limb gangrene and two had digit or extremity amputation. Severe intimal thickening of major arteries with thrombus formation was noted but no significant abnormality was found in the clotting activity of the blood. Histochemical studies in three cases suggested a reduction of fibrinolytic activity at the site of thrombosis in the diseased vessels. These studies support the view that the vascular system is profoundly altered in scleroderma and may play an important role in some manifestations of the disease.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/34. Scleroderma heart disease with slow flow velocity in coronary arteries.A young woman with scleroderma heart disease is presented. Complete work-up including hemodynamic studies revealed biventricular dysfunction, left ventricular hypokinesia and normal coronary arteries with slow flow velocity in coronary arteries. This finding, though not diagnostic, is consistent with small vessels disease secondary to scleroderma. Favorable prognosis in our patient on medical management is encouraging. No conclusions can be drawn on the basis of one patient. Further work is warranted in scleroderma patients with cardiomegaly to define the status of the myocardial microcirculation and its possible role in their prognosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/34. Scleroderma, stroke, optic neuropathy: a rare association.A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/34. The many faces of scleroderma.This review integrates the clinical aspects of systemic sclerosis (SSc; scleroderma) and scleroderma-like conditions with new knowledge of the control of blood vessel tone and the role of anoxia in the activation of connective tissues leading to fibrosis. serologic tests, high resolution computed tomographic scanning, bronchoalveolar lavage, and physiologic assessment of pulmonary gas diffusion are compared as diagnostic tools and as means of quantitating internal organ involvement. Treatment of Raynaud's disease and phenomenon, management of scleroderma renal crisis, and new means for improving gastrointestinal function with octreotide, the somatostatin analogue, also are discussed. The relationship between idiopathic forms of SSc and eosinophilic fasciitis/eosinophilia-myalgia syndrome caused by L-tryptophan ingestion and the scleroderma-like disease associated with silicone breast implants also is discussed.- - - - - - - - - - ranking = 4.7370954058064keywords = blood vessel, vessel (Clic here for more details about this article) |
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