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170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe the case of a patient of 22 with hypertension and livedo reticularis who, after presenting with a left brachial monoparesis became progressively demented over a period of five years and died at the age of 27 from a cerebro-meningeal haemorrhage. Angiographically, diffuse distal obliteration of the cerebral arteries was seen with deep networks of the moyamoya type involving the lenticulostriate arteries; similar changes were present in the upper left limb. Histopathological investigations showed obliterative thromboangiitis affecting not only the cerebrum, the brain stem and the cerebellum, but also the viscera. Analysis of this case and consideration of the theoretical possibilities leads the authors to urge that thromboangiosis together with its cerebral manifestations should be considered a nosological entity.
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PMID:[Anatomoclinical and angiographic study of a case of disseminated thromboangiosis with predominant cerebral manifestations]. 98 58

Two cases of Sneddon syndrome (S.s.) in a 33 and 53-year-old women who developed arterial hypertension, cerebral ischaemic signs and who have livedo reticularis or livedo racemosa, are reported. The authors describe clinical, radiological and biological features of this rare disease, as well as diagnostic investigations including the measurements of the antiphospholipid antibodies (APA). The possible role of APA in the pathogenesis of S.s. is discussed.
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PMID:[2 cases of Sneddon syndrome]. 140 98

Sneddon's syndrome refers to the rare association of extensive livedo reticularis with multiple ischaemic cerebrovascular episodes. Endarteritis obliterans is the most common cutaneous pathology. It is likely that several pathogenic mechanisms may give rise to Sneddon's syndrome, as the condition is associated with a high incidence of generalised atherosclerosis, hypertension, valvular heart disease and the presence of antiphospholipid antibodies.
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PMID:Sneddon's syndrome. 145 2

Sneddon syndrome is know as the association of idiopathic livedo reticularis and cerebrovascular lesions. The most characteristic trait of this syndromes is a non-inflammatory arteriopathy in medium caliber vessels. The pathogenic role of antiphospholipid antibodies in this disease is not clear. Clinical characteristics and etiopathogenic features of eight patients with Sneddon's syndrome are reviewed, specially regarding its relationship with primary antiphospholipid syndrome. A female predominance was found (3:1) as well as a relationship with hypertension (five patients suffered hypertension), but no relation was found with contraceptive use. Three patients showed evidence of antiphospholipid antibodies, present as anticardiolipin antibodies with significative titers in three cases and lupus anticoagulant in one. Digital artery biopsy performed in four patients showed in all of them the pathologic features characteristic of this disease. Seven patients were treated with platelet activity inhibitors and one with oral anticoagulants. Six of them have had a year and half follow-up without showing any new ischemic stroke. The main etiopathogenic factor on Sneddon's syndrome is the presence of a non-inflammatory arteriopathy in medium caliber vessels. Blood hypertension and antiphospholipid antibodies could play a role in the development of cerebrovascular lesions in some cases. No relationship has been found with oral contraceptives in this series of patients. Medium term prognosis with platelet activity inhibitors therapy seems benign.
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PMID:[Sneddon's syndrome: its clinical characteristics and etiopathogenic factors]. 846 57

A case of a 6 year old girl with angiographically and bioptically verified systemic polyarteritis nodosa is reported. Secondary hyperaldosteronism due to involvement of small and medium-sized renal arteries caused severe hypertension and left ventricular hypertrophy, and together with abdominal complaints, loss of weight, livedo reticularis and elevated titers of streptococcal antibodies formed the clinical pattern of the disease. It may be assumed, that there is a correlation between the recurrent streptococcal infections and the development of systemic polyarteritis nodosa by hypersensitivity or disordered immunologic reactivity. Therapy and short time follow up is reported. For treatment of hypertension an angiotensin converting encyme inhibitor was used, which immediately slowed down the maximal stimulated plasmarenin-angiotensin system.
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PMID:[Panarteritis nodosa with secondary hyperaldosteronism involving renal arterioles]. 167 49

Two cases of primary antiphospholipid syndrome are described. A girl presented with myocardial infarction at the age of 6. afterward developed chorea, livedo reticularis, thrombocytopenia and circulating lupus anticoagulant (LAC). A boy, age 7, had an episode of intracranial hypertension and a deep venous thrombosis of a lower left limb, both recurrent in the following years. A high titer of IgG anticardiolipin antibodies (aCI) was detected. These observations suggest that both LAC and aCI tests should be performed in children with thromboembolic phenomena when the criteria for a definite autoimmune disease are lacking.
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PMID:Primary antiphospholipid syndrome: a report of two pediatric cases. 192 Mar 12

A case of polyarteritis nodosa is reported. The patient, a 56-year-old white woman, had cutaneous nodules, ulcers and livedo reticularis over the limbs. Abdominal angiography revealed the presence of microaneurysms. Hypertension, rheumatic heart disease (under anticoagulation therapy) and diabetes mellitus, were also detected. The controversial attempt in distinguishing between systemic and cutaneous polyarteritis is emphasized, and the influence of warfarin on skin lesions morphology is discussed.
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PMID:[Polyarteritis nodosa. Considerations on a clinical case]. 198

Sneddon's syndrome consists of livedo reticularis and cerebral vascular accidents with no evidence of systemic disease responsible for the livedo. The syndrome has been assimilated to a subgroup of systemic lupus erythematosus (SLE) with presence of antibodies directed against phospholipids. Recently, a significant increase in the frequency of cardiac valve diseases has been demonstrated in some SLE patients with livedo reticularis, cerebral vascular accidents and antiphospholipid antibodies. We report the case of a 26-year old woman who had been presenting for 6 years with idiopathic livedo reticularis. Her history was remarkable for the occurrence of 2 cerebral ischaemic accidents at the ages of 23 and 26 years, generalized convulsive seizures at 22 years, and hypertension of pregnancy with 2 miscarriages. Biopsy of the livedo showed normal histological patterns, but electron microscopy detected an obliterating endothelial proliferation and endothelial cells with numerous Weibel-Palade bodies. Laboratory signs of SLE, as well as antiphospholipid antibodies were absent. At the age of 26 years, cardiac abnormalities were heard at auscultation for the first time, and echocardiography showed that they were due to a fairly loose mitral stenosis. According to Burton's criteria our patient had all the typical features of Sneddon's syndrome. The finding of mitral stenosis--an emboligenic cardiopathy that is potentially responsible for cerebral vascular accidents--raises the problem of its relationship with Sneddon's syndrome. The association does not seem to be fortuitous, since our case is very similar to the cases of SLE or antiphospholipid antibody syndrome associated with cardiac valve lesions. However, this case is particular in that 6 years after the onset of the disease there was still no sign of SLE and of antiphospholipid antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Livedo reticularis, cerebrovascular disorders and mitral disease: a new cause of Sneddon's syndrome?]. 208 86

The authors report the case of a young woman with Sneddon's syndrome and expose literature review. This rare entity is characterized by idiopathic livedo reticularis, ischemic stroke, and occasionally, mild arterial hypertension. Skin biopsy shows endarteritis obliterans of deep dermal arteries. The pathogenesis of this disorder is still unknown and the treatment is not clearly established.
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PMID:[Sneddon's syndrome: review of the literature apropos of a case]. 209 37

We studied 24 patients (18 women, 6 men), aged 29 to 54, with Sneddon's syndrome. The clinical picture of Sneddon's syndrome was characterized by cerebrovascular disorders, livedo reticularis, disturbance of peripheral circulation, arterial hypertension, cardiac pathology (ischemic heart disease, heart murmurs), complicated obstetric history in women, and disturbed sexual function in men. In 6 of 17 examined patients with Sneddon's syndrome there was a high concentration of anticardiolipin antibodies (ACA) but no antibodies to native DNA and LE cells. The course of the disease in the patients with a high ACA level, when compared with normal ACA level patients, was characterized by a more rapid progression and more severe clinical manifestations. The study demonstrates the similarity of clinical symptoms and immunologic disturbances in Sneddon's syndrome and the antiphospholipid syndrome and suggests the importance of ACA in the pathogenesis of some cases of Sneddon's syndrome.
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PMID:Anticardiolipin antibodies in Sneddon's syndrome. 231 89


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