1/16. Jejunal telangiectasias as a cause of massive bleeding in a patient with scleroderma.Telengiectasias (arteriovenous malformations) can be seen in scleroderma throughout the gastrointestinal tract, including the stomach, small bowel and colon. Massive gastrointestinal bleeding rarely results from these malformations in scleroderma. The case of a patient presenting with severe jejunal bleeding secondary to telangiectasias with special regard to the management is discussed. This case emphasizes the importance of endoscopic examination combined with mesenteric angiography in patients with scleroderma who present with a high index of suspicion of telangiectasias as a source of bleeding.- - - - - - - - - - ranking = 1keywords = scleroderma (Clic here for more details about this article) |
2/16. 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 = 0.14285714285714keywords = scleroderma (Clic here for more details about this article) |
3/16. 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 = 0.85714285714286keywords = scleroderma (Clic here for more details about this article) |
4/16. The role of the intercellular matrix in dermal calcinosis of the CRST syndrome. An electron-microscopic study.We performed an electron-microscopic study on calcium deposits in two patients with calcifying scleroderma (CRST syndrome). calcium deposits were detected both intracellularly in the mitochondria of phagocytic cells and extracellularly. Needle-like crystals measuring up to 4,500 A in length and approximately 60 A in width were present in both the Von Kossa-positive regions and the apparently normal dermal areas around the calcification sites. Although the fibrillar matrix's ultrastructure was normal, hollow oxytalan fibrils were detected. Slightly electron-opaque, star-shaped material was observed among the fibrillar component of the matrix (matrix granules), which is the ultrastructural expression of some types of proteoglycans containing keratan sulphate and chondroitin sulphate. These granules cannot be detected in normal dermis. The extrafibrillary calcium deposits on these mucopolysaccharide structures may represent an early event in the complex pathogenesis of calcification in the CRST syndrome.- - - - - - - - - - ranking = 0.14285714285714keywords = scleroderma (Clic here for more details about this article) |
5/16. Clinical association of autoantibodies to fibrillarin with diffuse scleroderma and disseminated telangiectasia.Circulating autoantibodies against a variety of nuclear and nucleolar antigens are characteristic serologic findings in systemic scleroderma. Some of these antibodies correlate with clinical subsets of the disease. We describe three patients with systemic scleroderma and high autoantibody titers against U3 ribonucleoprotein-associated fibrillarin, a recently identified 34 kD nucleolar protein. These patients showed a progressive course with multiple organ and diffuse skin involvement with disseminated telangiectasia.- - - - - - - - - - ranking = 0.85714285714286keywords = scleroderma (Clic here for more details about this article) |
6/16. Pulmonary hypertension in the crest syndrome: variant of systemic sclerosis (scleroderma)--a case report.The crest syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.- - - - - - - - - - ranking = 0.85714285714286keywords = scleroderma (Clic here for more details about this article) |
7/16. Endobronchial telangiectasias and hemoptysis in scleroderma.hemoptysis is considered a rare event in scleroderma and to date only two previous cases could be identified. The occurrence of hemoptysis with bleeding and friable telangiectasias is reported in a patient with rapidly progressing systemic sclerosis. This represents the first report of this association, although bleeding telangiectasias have been reported in other systems. A brief review of the relevant literature is included.- - - - - - - - - - ranking = 0.71428571428571keywords = scleroderma (Clic here for more details about this article) |
8/16. Appearance of a gastric ulcer during diphosphonate therapy in a woman with CRST syndrome.A 46-year-old woman with the CRST variant of scleroderma (calcinosis, Raynaud's phenomenon, sclerodactyly, and telangiectasis) was treated with a diphosphonate (disodium etidronate) in an effort to reduce her dystrophic calcifications or retard their progression. After 18 months of therapy, no improvement in her calcinosis was noted. In addition, she developed bloody diarrhea, epigastric burning, and weight loss during the last two months of Therapy. Upper gastrointestinal series revealed a gastric ulcer. endoscopy with biopsy proved it to be benign. Gastric ulcerations has been seen in animals given disodium etidronate but only at much higher doses than are currently recommended for humans. Although a cause and effect relationship cannot be established with certainty in this patient, it is suggested since she was not receiving other ulcerogenic drugs chronically. This information may be important to other investigators of disodium etidronate.- - - - - - - - - - ranking = 0.14285714285714keywords = scleroderma (Clic here for more details about this article) |
9/16. The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma).The crest syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the crest syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.- - - - - - - - - - ranking = 0.85714285714286keywords = scleroderma (Clic here for more details about this article) |
10/16. Cryoablation of drug-resistant ventricular tachycardia in a patient with a variant of scleroderma.A 37-year-old man with a benign variant of scleroderma (CRST syndrome: calcinosis circumscripta, Raynaud's phenomenon, sclerodactyly, and telangiectasia) presented with recurrent ventricular tachycardia. Preoperative electrophysiologic study suggested that the mechanism of tachycardia was an ectopic pacemaker focus in the right ventricle. Right ventricular dilatation, tricuspid insufficiency, normal pulmonary pressures, and normal coronary arteries were also demonstrated. At surgery, epicardial mapping localized the site of origin of ventricular tachycardia to the anterior right ventricle near the crista supraventricular. Intramural recordings of the site of tachycardia demonstrated autonomous activity unreflected on the peripheral ECG during brief periods of sinus rhythm. Local epicardial cooling of this area with a cryoprobe promptly terminated ventricular tachycardia with resumption of tachycardia on warming. The focus was ablated by freezing the area at -60 degrees C. The patient remained free of dysrhythmia on no anti-arrhythmic agents for eight months at which time he had a single recurrence of ventricular tachycardia from a different site in the right ventricle. This technique offers a method for ablating sites of dysrhythmia arising in diffusely diseased myocardium.- - - - - - - - - - ranking = 0.71428571428571keywords = scleroderma (Clic here for more details about this article) |
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