Cases reported "Calciphylaxis"

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1/37. Five cases of calciphylaxis and a review of the literature.

    calciphylaxis is a rare phenomenon of cutaneous necrosis that typically occurs in association with renal failure and has a poor prognosis. We report 5 new cases of calciphylaxis that illustrate the important clinical and histopathologic features of the disease. All patients had end-stage renal failure at the time that purpuric plaques and nodules were noted; these subsequently progressed to necrotic ulcers with eschars. All skin biopsy specimens showed varying degrees of calcification of the medial layer of blood vessel walls in the dermis and subcutaneous fat. Neither the product of serum calcium and phosphorus concentrations nor parathyroid hormone levels correlated temporally with the clinical observations in every case, emphasizing the importance of clinical-histopathologic correlation. Although certain features of calciphylaxis in humans resemble the animal model originally proposed, there are also some crucial differences. We review the pathogenesis, epidemiology, clinical and histopathologic features, and treatment of this disease.
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2/37. calciphylaxis associated with metastatic breast carcinoma.

    calciphylaxis is a rare disorder associated with calcification of small- and medium-sized blood vessels, and progressive skin necrosis usually seen in the setting of end-stage renal disease (ESRD) and secondary hyperparathyroidism. It has also been observed in primary hyperparathyroidism, hypercalcemia of malignancy (extensive bony metastasis of breast cancer), and an isolated case reported with end-stage liver disease. We report an unusual case of calciphylaxis associated with metastatic breast carcinoma in the absence of renal or parathyroid disease. calciphylaxis has generally been associated with end-stage renal disease and hyperparathyroidism. One previous case report described calciphylaxis occurring in a patient with metastatic adenocarcinoma of the breast and hypercalcemia. Our case represents the second reported case of calciphylaxis associated with osteolytic, metastatic breast cancer. Although ESRD with secondary hyperparathyroidism is the most common presentation of calciphylaxis, this case demonstrates that other conditions that alter normal calcium metabolism must be considered in the differential diagnosis.
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3/37. calciphylaxis.

    calciphylaxis is a rare, life-threatening condition of widespread metastatic calcification most commonly seen in the setting of end-stage renal disease. The etiology of calciphylaxis is not well described, though there are several hypotheses. Cutaneous lesions are characteristically found on the abdomen, buttocks, or thighs as reticulated, painful, purple plaques that often undergo ulceration and may serve as a portal of entry for potentially life-threatening infectious agents. histology reveals medial calcification with intimal proliferation involving small vessels in the subcutaneous fat, associated with a lymphohistiocytic infiltrate of the affected lobules. Treatment, including phosphate binders and parathyroidectomy, is not universally effective. We present one case of calciphylaxis and discuss the clinical features, pathophysiology, histology, and treatment of the condition.
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4/37. calciphylaxis in a patient with end-stage renal disease.

    calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification. It is seen almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. We experienced a case of 67-year-old man with calciphylaxis that manifested with characteristic skin lesions, pathologic findings, and laboratory changes. His skin lesions began as painful erythematous patches and subsequently progressed to necrotic ulcers with eschars on the distal aspect of the extremities. Pathologically, calcification was found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. His serum calcium was 9.5 mg/dL, phosphorus was 7.8 mg/dL, and nPTH was 99.9 pg/mL. The patient had been treated with surgical debridement and other supportive treatment. However, he eventually underwent an amputation below the right knee and died from sepsis.
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keywords = blood vessel, vessel
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5/37. calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K.

    calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin k deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. thrombosis and protein c deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein c deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.
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keywords = vessel
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6/37. calciphylaxis of the penis: a unique cause of Fournier's gangrene.

    calciphylaxis is a rare disease that is caused by calcium deposition in medium and small sized vessels of the skin causing ischemic necrosis. These lesions often become infected and cause septicemia. We report a unique case of penile and scrotal calciphylaxis that rapidly progressed to Fournier's gangrene. We review the etiology of calciphylaxis and also its management.
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keywords = vessel
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7/37. calciphylaxis in two patients with end-stage renal disease.

    Fatal calciphylaxis (CPX) occurred in two 71-year-old females both requiring haemodialysis for end-stage renal disease. Case 2 also had an associated follicular lymphoplasmocytoid lymphoma. Although laboratory tests disclosed normal coagulation parameters, this woman had a striking cutaneous histological picture of vessel thrombosis and finally died of disseminated intravascular coagulation. CPX is a rare but potentially life-threatening complication of renal failure. The clinical picture is primarily characterized by livedoid purpura with subsequent cutaneous ischaemia and painful ulcerations. Cutaneous ischaemic phenomena are sustained by a progressive process of vascular calcification and thrombosis involving small to medium size arteries of the dermis and subcutis. Although not yet clearly explained, the pathogenetic role of a predisposing hypercoagulability state is currently the most frequently considered hypothesis.
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keywords = vessel
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8/37. Intestinal ischemia and peripheral gangrene in a patient with chronic renal failure.

    Gastrointestinal complications are common in patients with renal failure and result in significant morbidity and mortality. Systemic calciphylaxis is an uncommon complication of renal failure, characterized by disseminated intravascular calcification and associated with progressive vascular compromise. We describe the case of a 63-year-old woman who presented with abdominal pain, elevated transaminases, and skin manifestations consistent with a vasculitic process. hand films and skin biopsies showed extensive vascular calcification, and a computerized tomography scan confirmed colonic perforation and disseminated visceral vascular calcification. Histologic analysis of the resected skin and colonic tissues revealed extensive ischemic damage and mural calcification of medium to large vessels. Gastrointestinal involvement has been reported in only 3 prior cases of calciphylaxis; consequently, gastroenterologists are often unaware of this disease entity and may fail to recognize it, even in patients with the classical presentation. Prompt diagnosis is crucial, as parathyroidectomy may result in clinical improvement in up to two thirds of patients who present with elevated parathyroid hormone levels.
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keywords = vessel
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9/37. calciphylaxis in a patient with rheumatoid arthritis without renal failure and hyperparathyroidism: the possible role of long-term steroid use and protein s deficiency.

    calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification that is seen almost exclusively in patients with end-stage renal disease and hyperparathyroidism. We report a case of calciphylaxis that may very well be due to a long-term steroid use in a rheumatoid arthritis patient who had neither end-stage renal disease nor hyperparathyroidism. We also discuss the possible role of protein s deficiency as a contributing factor.
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keywords = vessel
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10/37. Three-dimensional analysis of a calciphylaxis plaque: clues to pathogenesis.

    BACKGROUND: calciphylaxis is a rare, life-threatening disorder associated with chronic renal failure, presenting with ulcerating plaques leading to death by sepsis in 60% of patients. Calcification of subcutaneous arterioles, thromboses, and extravascular calcification have been demonstrated in incisional biopsy specimens. However, the sequence of these pathologic events is unknown. OBJECTIVE: We examined a calciphylaxis plaque to document the wave of pathologic change from its center to its periphery. methods: A calciphylaxis plaque was excised postmortem from a female patient. It was examined histologically along 12 radii from the center of the lesion to its periphery. RESULTS: Calcification of small subcutaneous vessels was present in all histologically abnormal sections and extended further peripherally than extravascular calcification by up to 3.0 cm and further than subcutaneous thrombosis by up to 1.5 cm. CONCLUSION: Vascular mural calcification is an early and essential process in the development of a calciphylaxis plaque.
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ranking = 0.11342567281416
keywords = vessel
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