1/9. Familial Sneddon's syndrome.A syndrome associating livedo reticularis (LR) with Cerebrovascular disease (CVD) was described, in 1965, by Sneddon. It occurs sporadically, but a few familial cases of Sneddon's Syndrome (SS) have been reported, like these 3 cases that represent one of the largest number among siblings. We studied three male brothers, aged 28, 37 and 42 years, with CVD (ischaemic stroke in 2 patients and cerebral haemorrhages in the third) and their sister with no CVD. All patients presented with long lasting livedo reticularis, extending beyond the lower limbs. skin biopsy on the centre of the reticular pattern showed, only in the second patient, partial endothelium detachment in dermo-hypodermic blood vessels. The males also had accesses of Livedoid vasculitis (LV), in which a skin biopsy showed obliteration of several upper dermal vessels with hialin thrombi and a very scarce inflammatory infiltrate. Complementary studies, with an extensive investigation on pro-coagulation/pro-thrombotic features including antiphospholipid antibodies, were repeatedly negative. Their non-consanguineous parents were not affected, but among these kindred of 9 individuals, apart from the 4 patients reported above, LR and LV were present in two other brothers and also in an aunt and uncle, suggesting autosomal dominant pattern of inheritance, with incomplete penetrance. The relationship between Sneddon's Syndrome and Antiphospholipid Antibody Syndrome is controversial. The present cases, having repeatedly negative antiphospholipid antibodies, support the classification of Sneddon's Syndrome as an independent nosological entity.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/9. Sneddon's syndrome associated with anticardiolipin antibody: a case report.We report the case of a young man suffering from the rare combination of livedo reticularis and recurrent ischemic cerebrovascular disease (Sneddon's syndrome). He also had a circulating anticardiolipin antibody. in the absence of systemic lupus erythematosus, we suggest the likelihood of a primary antiphospholipid syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/9. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/9. Sneddon's syndrome: generalized livedo reticularis and cerebrovascular disease. Importance of hemostatic screening.We report two cases of Sneddon's syndrome. Both cases had widespread livedo reticularis with repeated cerebrovascular accidents without persistent neurological deficit. In one case, hemostatic examination revealed an imbalance of plasminogen activator-inhibitor values, possibly related to the thrombogenic propensity of the syndrome. Treatment with acetylsalicylic acid led to normalization of hemostatic parameters and resulted in a symptom-free period of more than 10 months. The importance of hemostatic screening in patients with Sneddon's syndrome is discussed.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
5/9. Renal cholesterol embolism in patients with carotid stenosis: a severe and underdiagnosed complication following cerebrovascular procedures.Here, we report two cases with rapidly progressive renal failure, caused by cholesterol crystal embolism (CCE), after an angiography for carotid artery stenosis. The diagnosis was determined by histological examination and from clinical symptoms, including livedo reticularis and eosinophilia. Neurologists and neuroradiologists tend to underdiagnose CCE, which results from the same atherosclerotic risk factors as cerebrovascular disease. We need to understand more about CCE and identify its unique clinical symptoms to enable an early diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/9. Diffuse dermal angiomatosis of the breast.BACKGROUND: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. OBSERVATIONS: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved. One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. CONCLUSIONS: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxia may improve the patient's condition.- - - - - - - - - - ranking = 3keywords = vascular disease (Clic here for more details about this article) |
7/9. A large ulcer and cutaneous small-vessel vasculitis associated with syphilis infection.Cutaneous vasculitis (CV) is a condition with cutaneous manifestations and possible systemic involvement. The causative factors or associated diseases are usually drugs, infection, collagen vascular disease, or malignancy. syphilis as a cause of cutaneous vasculitis is rare. We report the case of a large cutaneous ulcer and small-vessel vasculitis associated with syphilis infection. We suggest that in apparently idiopathic CV or a chronic ulcer refractory to treatment, screening should be performed to detect any underlying infection such as syphilis. It is important to have a rapid and accurate diagnosis because the lesions are very contagious, but may be rapidly and completely cured by early administration of antibiotic treatment.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/9. Kohlmeier-Degos's disease with primary intestinal manifestation.Disseminated intestinal and cutaneous thromboangiitis (synonyms: Kohlmeier-Degos's syndrome, malignant atrophic papulosis, progressive arterial mesenterial vascular occlusive disease) is a rare, systemic vascular disease that is mainly manifested in the skin, gastrointestinal tract, and nervous system. The disease first appears as a necrotizing papulous dermatosis; as it generalizes, infarcted necroses develop in internal organs. These ischemic complications are the reason for the usually fatal outcome of the disease. A case report of a primary intestinal manifestation of this disease illustrates the clinical course, diagnosis, histopathologic findings, and differential diagnosis, with consideration of the current literature. Deposits of acid mucopolysaccharides and humoral immune mechanisms appear to play a role in the etiology and pathogenesis of this usually fatal vascular disease.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
9/9. Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases.Peripheral ulcerative keratitis (PUK) is a complication of collagen-vascular diseases such as rheumatoid arthritis (RA) and other systemic vasculitides. We report three cases of erythema elevatum diutinum with PUK. These patients presented with nodules and plaques consistent with erythema elevatum diutinum on the extremities and crusted or ulcerated purpuric lesions on the soles. Histopathological examination of these lesions revealed a dense neutrophilic infiltrate with nuclear dust and fibrin around blood vessels. All the patients developed PUK concomitant with the development of the skin lesions. The rheumatoid factor was positive at high titre in all three patients; this was associated with probable RA in one. Cutaneous lesions were dramatically improved by administration of dapsone in all cases. dapsone was also effective in treating the ocular lesions in two patients.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |