1/52. Cutaneous histiocytic lymphangitis: an unusual manifestation of rheumatoid arthritis.Two cases are presented of unusual cutaneous lesions associated with rheumatoid arthritis in underlying joints. The lesions were evanescent, erythematous and violaceous partly macular and partly indurated plaques, with a livedo-like pattern of erythema at the edge in one case. Histological changes were identical in the two cases. The major features were dilated, dermal lymphatics containing aggregates of inflammatory cells, mainly histiocytes, with adjacent perivascular lymphoid aggregates. An appropriate name for this reaction would appear to be cutaneous histiocytic lymphangitis.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
2/52. Sneddon's syndrome: a cause of cognitive decline in young adults.OBJECTIVE: To review the clinical, psychometric, laboratory, and radiologic findings of 6 patients with Sneddon's syndrome (SS) who presented with cognitive dysfunction rather than stroke. DESIGN AND methods: Case series. All patients fulfilled were diagnosed as SS based on the co-occurrence of livedo racemosa and neurologic disease. patients presenting with clinical stroke were excluded. RESULTS: patients presented with cognitive complaints at an early age and all noted skin lesions from 6 months to 10 years before onset of cognitive symptoms. Associated systemic disorders included hypertension and seizures. Laboratory evidence of a hypercoagulable condition was identified in 4 of 6 cases. brain MRI scans demonstrated atrophy, especially in parieto-occipital regions and cerebral blood flow on brain SPECT scan was reduced in a similar distribution. CONCLUSION: patients with SS can develop dementia without antecedent clinical stroke. While the specific pathogenic mechanism of dementia in SS remains speculative, the disease predominantly injures brain tissue in vascular "watershed" territories.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
3/52. An unusual case of calciphylaxis.BACKGROUND: Cutaneous calciphylaxis is a rare disorder that occurs most frequently in patients with end-stage renal disease (ESRD), those on hemodialysis, and renal transplant recipients. It is frequently associated with hyperparathyroidism and a markedly elevated calcium-phosphate product, and it carries a high mortality rate. The usual clinical presentation is of painful, stellate necrosis of the thighs or buttocks, often in the setting of livedo reticularis. death usually results from septicemia. OBJECTIVE: This report documents an unusual case of recurrent, self-limiting calciphylaxis in the setting of a patient with ESRD and discusses the clinical and pathologic features of this potentially very fatal disorder. methods AND RESULTS: A 52-year-old woman presented with a greater than one-year history of relapsing and remitting, exquisitely painful, necrotic, numular plaques on the abdomen, breast, and arm. This patient had a markedly elevated calcium-phosphate product and parathyroid hormone level. The diagnosis of calciphylaxis was made by wedge biopsy of the most recent plaque, revealing calcification of medium-sized subcutaneous vessels and lobular capillaries with associated epidermal necrosis. CONCLUSIONS: This case demonstrates an unusual clinical variant of calciphylaxis that presented without the characteristic stellate necrosis or livedo reticularis that normally marks this condition and spontaneous resolution without incurring septicemia. Regardless of morphology, calciphylaxis should be considered in the differential diagnosis of painful, necrotic lesions occurring in the setting of ESRD.- - - - - - - - - - ranking = 20.713307849675keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
4/52. Unusual manifestations of primary cutaneous amyloidosis in association with Raynaud's phenomenon and livedo reticularis.A patient with unusual manifestations of primary cutaneous amyloidosis, including macules, papules, oedematous plaques and urticarial lesions is described. Raynaud's phenomenon and livedo reticularis were an associated finding. During the acute phase, single doses of systemic corticosteroids resulted in an impressive, long-lasting improvement in cutaneous manifestations.- - - - - - - - - - ranking = 51.783269624189keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
5/52. Subcutaneous granuloma pyogenicum in patients with antiphospholipid antibodies.We describe 2 cases of subcutaneous granuloma pyogenicum occurring in patients with antiphospholipid antibodies. The 2 patients had asymptomatic nodules with histopathological findings of a typical subcutaneous granuloma pyogenicum. In addition, case 1 was diagnosed as primary antiphospholipid syndrome with livedo reticularis, superficial thrombophlebitis, thrombocytopenia, cerebral and pulmonary infarcts and renal involvement. Case 2 had no clinical manifestations of vascular occlusive diseases, although anticardiolipin antibody levels were highly positive. To our knowledge, these are the first reported cases of subcutaneous granuloma pyogenicum associated with antiphospholipid antibodies. Antiphospholipid-antibody-mediated thrombosis or immunological mechanisms may be involved in subcutaneous granuloma pyogenicum in our cases.- - - - - - - - - - ranking = 10.356653924838keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
6/52. Hemostatic abnormalities in Sneddon's syndrome.Sneddon's syndrome is a rare condition comprising widespread livedo retucularis and multiple episodes of transient cerebral ischemia. Treatment to date has been empirical. The hemostatic/thrombotic status of 4 patients with Sneddon's syndrome was studied by a unique technique, hemostatometry, which measures primary hemostasis (shear-induced platelet plug formation), the overall coagulation, and thrombolysis (dislodgment of the hemostatic plugs) from nonanticoagulated blood. In all 4 patients, platelet reactivity, which shows itself in the initial phase of the hemostatic reaction, was enhanced. The overall hemostasis, in which the generation of thrombin by activated platelets plays the decisive role, was enhanced in 3 patients. Three of the 4 patients had hypercoagulation, and in 3, spontaneous thrombolysis was inhibited. Treatment was commenced with aspirin and nifedipine, and patients were monitored both clinically and by serial hemostatometry over two years. One patient had one further transient ischemic episode; the other 3 remained asymptomatic. Thus, the observed clinical improvement correlated with improvement of the hemostatic profile.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
7/52. Sneddon's syndrome and primary antiphospholipid syndrome: a case report.The association between livedo reticularis and cerebrovascular accidents is known as Sneddon's syndrome. We describe a case in which Sneddon's syndrome appeared as a clinical manifestation of primary antiphospholipid syndrome.- - - - - - - - - - ranking = 10.356653924838keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
8/52. Widespread cutaneous and systemic calcification (calciphylaxis) in patients with the acquired immunodeficiency syndrome and renal disease.BACKGROUND: calciphylaxis is a form of widespread calcification that may occur in patients with renal disease and hyperparathyroidism. The skin is often affected secondary to vascular compromise and ischemia. patients infected with the human immunodeficiency virus (hiv) are predisposed to renal failure and thus may develop this serious complication. OBJECTIVE: The purpose of this article is to describe two cases of fatal calciphylaxis in patients with the acquired immunodeficiency syndrome and renal disease. methods: Two patients were examined clinically and through the performance of serologic studies and skin biopsies. RESULTS: Both patients had a widespread livedo reticularis-like eruption, which rapidly progressed to cutaneous gangrene. Patient 1 had elevated serum calcium and phosphorus levels whereas patient 2 had normal values but had markedly elevated serum parathyroid hormone levels. biopsy specimens in both cases showed vascular thrombosis and soft tissue calcification. CONCLUSION: Widespread calcification may develop in patients with hiv infection and renal failure and, in some cases, calcium and phosphorus levels may be normal.- - - - - - - - - - ranking = 10.356653924838keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
9/52. Amyloid elastosis: analysis of the role of amyloid P component.We report the second case of amyloid elastosis. Our patient had an underlying primary systemic amyloidosis with lambda light chain paraproteinemia. Salient clinical features included a sclerodermatous facial appearance, cordlike thickening of superficial blood vessels, neck skin resembling that in pseudoxanthoma elasticum, livedo reticularis-like changes on the trunk, Raynaud's phenomenon, arterial and venous thromboses, and the nephrotic syndrome. Amyloid deposits were present in the dermis, around appendages, in blood vessel walls, and in a striking distribution surrounding individual elastic fibers, that appeared shortened and fragmented. Immunofluorescence, electron microscopic, and immunoultrastructural studies with antibodies to lambda light chain, localized the amyloid deposits to the region of the elastic fiber microfibrils, with which amyloid P component (AP) is invariably associated in normal tissues. Because AP binds amyloid fibrils, codistribution of amyloid deposits and AP in amyloid elastosis strongly supports the theory that elastic fiber-associated AP may act as a nidus for amyloid deposition.- - - - - - - - - - ranking = 10.356653924838keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
10/52. Diffuse dermal angiomatosis.Diffuse dermal angiomatosis (DDA) is an acquired, benign vascular proliferation characterized clinically by poorly circumscribed, violaceous, livedoid plaques with frequent ulceration. Histologically, a diffuse interstitial proliferation of CD31-positive endothelial cells is present within the papillary and reticular dermis. Endothelial atypia, atypical mitoses, and vasculitis are lacking. We describe a case of DDA in a 53-year-old man with peripheral vascular atherosclerosis that resolved following revascularization. Early correction of the associated ischemic peripheral vascular disease promotes resolution of this unusual clinicopathologic entity.- - - - - - - - - - ranking = 1keywords = livedo (Clic here for more details about this article) |
| | Next -> |