1/7. Generalized essential telangiectasia in a patient with Graves' disease: should the spectrum of autoimmune diseases associated with generalized telangiectasia be expanded?Generalized essential telangiectasia (GET), as originally described, is not associated with any underlying disease. Although patients with GET lack the typical periungual telangiectases associated with autoimmune collagen vascular diseases, these patients may have an underlying autoimmune process. We present a patient with a history of Graves' disease and low-titer anti-nuclear antibodies, who developed rapidly progressive generalized telangiectases. The gender and age of the majority of patients with GET fit well within the demographics of most autoimmune diseases. The documented occurrence of an autoimmune disease in several of the limited number of patients previously diagnosed with GET provides additional evidence that GET may be associated with an underlying autoimmune disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/7. Severe visual loss associated with retinal telangiectasis and facioscapulohumeral muscular dystrophy.Facioscapulohumeral (FSH) muscular dystrophy is known to be associated with retinal telangiectasis. However, there are only few reports of severe visual loss due to exudative complications, so the risk to vision has not been established. Because of the possible therapeutic implications, we have described two cases of young girls who developed FSH muscular dystrophy and exudative retinal detachment due to telangiectasis. In the first patient, the severity of the disease precluded visual recovery despite extensive photo- and cryotherapy. In the other, visual acuity in both affected eyes was retained after treatment. Fundus examinations in young children at risk of having the gene for FSH muscular dystrophy may be justified so that retinal vascular disease can be detected before it becomes untreatable.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/7. Group 2A idiopathic juxtafoveolar retinal telangiectasia in monozygotic twins.PURPOSE: To report the clinical and fluorescein angiographic characteristics of monozygotic twins affected by group 2A idiopathic juxtafoveolar retinal telangiectasia. DESIGN: Observational case report. methods: Both eyes of identical twins were examined in a clinical practice setting. RESULTS: Two 68-year-old Caucasian, monozygotic, female twins were examined. In one twin, visual acuity was 20/50 in the right eye (OD) and 20/40 in the left (OS). ophthalmoscopy demonstrated right-angle venules without edema in both eyes (OU). fluorescein angiography demonstrated areas of leakage temporal to the fovea OU with some nasal leakage OS. In her identical twin sister, with type II diabetes, visual acuity was 20/60 OD and 20/25 OS. Right-angle venules OU, refractile deposits OS, and no macular edema OU were present. fluorescein angiography demonstrated areas of parafoveal leakage temporal and nasal OD and temporal OS. CONCLUSION: This is the second set of female monozygotic twins with group 2A idiopathic juxtafoveolar retinal telangiectasia reported in the literature. This finding suggests a genetic component in the pathogenesis of this retinal vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/7. Optical coherence tomography evaluation of macular edema after intravitreal triamcinolone acetonide in patients with parafoveal telangiectasis.PURPOSE: Parafoveal telangiectasis (PT) is a developmental or acquired microvascular abnormality of the macula. Leakage of the abnormal parafoveal capillaries leads to macular edema with subsequent decrease in visual acuity. Intravitreal triamcinolone acetonide is recently widely used in the management of intraocular proliferative, edematous, and neovascular diseases. methods: This report presents the evaluation of three cases with PT in whom intravitreal triamcinolone acetonide (IVTA) injection was performed. Fundus fluorescein angiography (FA) and optical coherence tomography (OCT) were used in follow-up of the patients. RESULTS: Following pars plana intravitreal injection of 4 mg of triamcinolone acetonide, the patients had angiographic improvement of the macular edema and minimal decrease in retinal thickness on OCT, accompanied by improvement in visual acuity and subjective visual assessment. CONCLUSIONS: The results of the present study on parafoveal capillary telangiectasis suggest that the intravitreal injection of triamcinolone acetonide may be a therapeutic option to increase visual acuity and decrease vascular leakage on FA. Following IVTA procedure, follow-up of these patients with both OCT and FA is important for correct clinical evaluation. Future studies on this method seem to be warranted.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/7. iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis.We describe a case of iris microhaemangiomas (iris vascular tufts) causing spontaneous hyphaema and where examination showed that the patient also had idiopathic juxtafoveolar retinal telangiectasis. A systematic search through pubmed, EMBASE and reference lists revealed a total of 90 reported cases of iris microhaemangiomas. To our knowledge, no previous reports have described concurrent findings of iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis in a patient. Both conditions are acquired vascular diseases, but their pathogenesis and aetiologies are unknown. iris microhaemangiomas are important clinically because they are occasionally a cause of spontaneous hyphaema and may induce transient elevation of intraocular pressure.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/7. Nodular regenerative hyperplasia of the liver, crest syndrome and primary biliary cirrhosis: an overlap syndrome?Nodular regenerative hyperplasia of the liver (NRHL) has been found in association with collagen vascular diseases, after drug therapy, with autoimmune disease, and with a variety of haematological disorders. The association of NRHL with the syndrome of calcinosis cutis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia (crest syndrome) has only been reported on two previous occasions. The liver disease usually associated with crest syndrome is primary biliary cirrhosis (PBC) and recently nodular hyperplasia of the liver has been reported in patients with early stage PBC. We present a case of NRHL with crest syndrome and serological and biochemical features of PBC, a newly recognised overlap syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/7. Large vessel occlusive disease associated with crest syndrome and scleroderma.OBJECTIVES--To report the cases of three patients with crest syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. methods--The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS--These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with crest syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS--These cases suggest an association of both the crest syndrome and scleroderma with macrovascular disease.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |