1/5. calciphylaxis, a poorly understood clinical syndrome: three case reports and a review of the literature.Systemic calcification syndromes are a recognized complication occurring in some patients with end-stage renal disease (ESRD) and secondary hyperparathyroidism. These patients develop severe livedo reticularis and subcutaneous tissue lesions progressing to frank necrosis and ultimately large areas of eschar. Clinically this syndrome is known as calciphylaxis; these lesions are usually resistant to aggressive debridement, systemic antibiotics, and revascularization procedures. We report three patients with somewhat different clinical presentations but all sharing a common link of exquisitely painful leg ulcers initially being treated as ischemic lesions or venous stasis-type ulcerations. These three patients were diagnosed with calciphylaxis on the basis of clinical, biochemical, and histopathological criteria. Two patients underwent parathyroidectomy late in the progression of their disease, with some resolution of their ulcerative lesions.- - - - - - - - - - ranking = 1keywords = calciphylaxis (Clic here for more details about this article) |
2/5. 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 = 4keywords = calciphylaxis (Clic here for more details about this article) |
3/5. Bone scintigraphy of calciphylaxis: a syndrome of vascular calcification and skin necrosis.calciphylaxis is a highly morbid syndrome of vascular calcification and skin necrosis, the pathophysiology of which remains largely elusive. We report a patient with end-stage renal disease and multiple painful skin lesions who underwent a bone scan for extremity pain. Increased tracer accumulation was seen in the subcutaneous tissues of the trunk and lower extremities. In this case, the bone scan aided in the diagnosis and treatment of calciphylaxis for a patient who experienced a relatively short hospital stay.- - - - - - - - - - ranking = 2.5keywords = calciphylaxis (Clic here for more details about this article) |
4/5. Post-renal transplant calciphylaxis: successful treatment with parathyroidectomy.calciphylaxis occurs in renal transplant patients more frequently than in chronic hemodialysis patients. It can occur in a patient with normal renal function who is also normocalcemic. Early parathyroidectomy can lead to healing of the skin lesions and prevent death of sepsis.- - - - - - - - - - ranking = 2keywords = calciphylaxis (Clic here for more details about this article) |
5/5. Systemic calciphylaxis revisited.A syndrome characterized by rapidly progressive ischemic necrosis involving large areas of the skin and muscle, and by peripheral gangrene associated with extensive vascular calcifications was observed in a patient with end-stage renal failure on chronic hemodialysis. In an effort to control the disease, parathyroidectomy was performed which resulted in rapid improvement of tissue perfusion. However, the patient eventually died from sepsis within 2 months after admission. This case presents the typical features of the syndrome of systemic calciphylaxis. The literature is reviewed searching for similar cases of this poorly recognized, but life-threatening, clinical syndrome. The pathogenesis, clinical manifestations, and therapy of this unusual and rapidly progressive, but potentially reversible, condition are reviewed with emphasis on its prompt recognition and appropriate management.- - - - - - - - - - ranking = 2.5keywords = calciphylaxis (Clic here for more details about this article) |