1/5. Typical features of calciphylaxis in a patient with end-stage renal failure, diabetes mellitus and oral anticoagulation.We report a multimorbid patient with end-stage renal failure showing a large necrosis and livedo racemosa on the right thigh. histology revealed medial calcification of the small arteries typical of calciphylaxis. We found the typical features of the disease with different risk factors like elevated calcium-phosphate product, diabetes mellitus and oral anticoagulation. On account of the location of the skin lesions, a bad prognosis was expected. In spite of therapeutical measures with lowering of the calcium and phosphate levels, the patient died 1 month after the diagnosis had been made.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/5. calciphylaxis with peau d'orange induration and absence of classical features of purpura, livedo reticularis and ulcers.calciphylaxis is an ill-defined syndrome that is commonly associated with chronic renal failure. Its heterogeneous clinical features include painful livedo reticularis-like purpuric patches and plaques, vesicles, irregularly shaped ulcers, and black eschars. Despite demonstration of extensive vascular arteriolar calcification in this syndrome, its exact pathogenesis remains unknown. Here, we report a case of calciphylaxis presenting with indurated plaques without the usual clinical picture of livedo reticulate purpura, ulcers or necrotic eschars. This case provides an opportunity to review the clinical spectrum of calciphylaxis and to discuss the therapeutic approaches and pathogenesis of this syndrome from deep intra-wall vascular calcification to the resulting infarctions of adjacent tissues.- - - - - - - - - - ranking = 0.4keywords = calciphylaxis (Clic here for more details about this article) |
3/5. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.- - - - - - - - - - ranking = 1.6keywords = calciphylaxis (Clic here for more details about this article) |
4/5. calciphylaxis: early recognition and management.calciphylaxis, a syndrome of disseminated calcification found in chronic renal failure patients with secondary hyperparathyroidism, results in soft tissue calcification and vascular medial calcinosis leading to subsequent ischemic tissue necrosis. It is a rarely occurring condition in which patients present with painful, violaceous, mottled lesions of the extremities and/or trunk that progress to skin and subcutaneous tissue necrosis, non-healing ulcers, and gangrene. We reviewed the clinical course of seven patients (aged 24-69) with calciphylaxis treated at our institution over a 4-year period (October 1988-June 1992). All seven patients underwent parathyroidectomy, with a mean time of 8 weeks (range 3-20 weeks) between the onset of calciphylactic symptoms and parathyroidectomy. Four patients died, three secondary to wound-related sepsis. Of the three survivors, two healed soft tissue lesions primarily. The other required extremity amputation and wound excision before healing. Neither anatomical location of the soft tissue lesions nor post-parathyroidectomy serum calcium and phosphorus levels had any bearing on wound healing or mortality. Lesion severity at the time of parathyroidectomy appeared to best correlate with clinical course. Although treatment with phosphate-binding antacids, total or subtotal parathyroidectomy, and avoidance of challengers such as vitamin d or local tissue trauma remain the mainstays of therapy, the uniform cure for calciphylaxis remains elusive. prognosis for patients with calciphylaxis is dismal, even following late surgical intervention. Earlier recognition of the signs and symptoms of calciphylaxis should lead to timely parathyroidectomy in the hopes of ameliorating the symptoms and preventing or retarding its progressive sequelae.- - - - - - - - - - ranking = 0.8keywords = calciphylaxis (Clic here for more details about this article) |
5/5. Extensive necrotizing livedo reticularis in a patient with chronic renal failure, hyperparathyroidism and coagulation disorder: regression after subtotal parathyroidectomy.Necrotizing livedo reticularis is an infrequent, life-threatening complication of chronic renal failure. Since Selye's studies in 1962, calciphylaxis, i.e. acute calcium deposition in tissue, is considered the main pathomechanism, especially because hyperparathyroidism are very frequently present. However, other etiological and/or triggering factors, such as coagulation disorders, direct cellular toxicity of parathormone or calcium on endothelium, might be involved, acting perhaps in a cumulative way. We report a case with a circulating anticoagulant which supports this hypothesis.- - - - - - - - - - ranking = 0.2keywords = calciphylaxis (Clic here for more details about this article) |