1/12. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study.We report a 54-year-old male, with a 5-year history of spreading asymptomatic generalized cutaneous telangiectases. The patient had no mucosal or nail involvement, no positive family history and no clinical evidence of systemic disease or bleeding diathesis. Histologically, the superficial small dermal blood vessels were dilated and showed thickened walls with hyaline perivascular material, staining as collagen. The vessel walls were PAS and colloidal iron stain positive, and immuno-histochemically lacked actin staining. collagen IV, fibronectin and laminin antibodies showed the material deposited around the basement membrane zone. Ultrastructurally, the vessels were post-capillary venules (PCV) and showed marked collagen deposition around the basal lamina. There were many abnormally banded widely spaced fibres with 100-150 nm periodicity (Luse bodies), in addition to regular banded collagen. pericytes were sparse and lacked intracytoplasmic filaments, and few veil or fibroblastic cells were seen embedded within the collagen. We believe this is a form of cutaneous microangiopathy not previously described, with distinct morphology and unique ultrastructural features. It may be due to a genetic defect with erroneous production of disorganized collagen in the cutaneous microvasculature. Dermatologists and Dermatopathologists should be aware of this unusual cutaneous vasculopathy.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
2/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
3/12. Retinal angiopathy and polypoidal choroidal vasculopathy.PURPOSE: To describe the clinical and angiographic features of patients with polypoidal choroidal vasculopathy, exudative detachment of the macula, and an associated retinal microangiopathy. methods: Case series. RESULTS: Four patients with chronic exudative detachment of the macula with a variable degree of lipid deposition are described. The retina in the detached area, but not beyond, was noted to have a microangiopathy. There was capillary telangiectasia, microaneurysm formation, patchy nonperfusion, and intraretinal leakage. In each patient, there were no other retinal vascular changes in the fundus of either eye. The fluorescein angiogram showed subretinal leakage suspicious for occult choroidal neovascularization. The indocyanine green angiogram showed the presence of underlying polypoidal choroidal neovascularization, accounting for the exudative detachment. After photocoagulation, the retinal angiopathy improved, but not completely. CONCLUSION: Retinal microangiopathy may occur in a chronic macular detachment secondary to polypoidal choroidal neovascularization. The development of these secondary retinal changes is not clearly understood; however, hypoxia from the chronic detachment, a neurotoxic effect from the lipid deposition, or a biochemically induced microvascular abnormality from secretion of vasogenic mediators are possible mechanisms. indocyanine green angiography is helpful in making a definitive diagnosis. Clinicians should be aware that a retinal microangiopathy may occur in such eyes so that the proper diagnosis can be made and appropriate treatment administered.- - - - - - - - - - ranking = 4keywords = microangiopathy (Clic here for more details about this article) |
4/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
5/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
6/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
7/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
8/12. 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 = 0.15457038451577keywords = vascular disease (Clic here for more details about this article) |
9/12. Parafoveal telangiectasis and diabetic retinopathy.Five patients with mild nonproliferative diabetic retinopathy had visual loss associated with parafoveal telangiectasis. Minimal macular edema with characteristic parafoveal plaques of subretinal pigment epithelial hyperplasia was seen in all patients. fluorescein angiography revealed the presence of ectatic, dilated, leaking perifoveal capillaries. The occurrence of parafoveal telangiectasis in patients with diabetic retinopathy has, to our knowledge, not been previously described. One previous clinicopathologic report described the histologic appearance of parafoveal telangiectasis in a nondiabetic patient to be similar to that seen in diabetic patients. The association of parafoveal telangiectasis and diabetic retinopathy raises interesting speculations into the pathogenesis of the entity of parafoveal telangiectasis. In addition, the importance of obtaining fluorescein angiography prior to therapy of diabetic macular edema is emphasized.- - - - - - - - - - ranking = 0.60007269102105keywords = diabetic (Clic here for more details about this article) |
10/12. Tractional venous loops in diabetic retinopathy.A venous loop developed in a patient with proliferative diabetic retinopathy. fluorescein angiography showed staining of the vessel wall and nonperfusion of the surrounding capillary bed. light microscopy and scanning and transmission electron microscopy showed the loop to consist of a telangiectatic retinal vein that had passed through a discontinuity in the internal limiting membrane. The thin-walled vessel loop was lined by an attenuated endothelium and occasional pericytes. The vitreous was detached posteriorly except at the venous loop and a few other points at which there was tenting of the internal limiting membrane. These findings suggested that vitreous traction plays a role in the pathogenesis of these venous loops.- - - - - - - - - - ranking = 0.30003634551053keywords = diabetic (Clic here for more details about this article) |
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