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PubMed2001

Painful ulcers in calciphylaxis - combined treatment with maggot therapy and oral pentoxyfillin.

BACKGROUND: Calciphylaxis is a rare syndrome developing predominantly in female patients suffering from end-stage renal disease (ESRD) with secondary hyperparathyroidism. Skin lesions begin as superficial patches that quickly progress to painful necrotic ulcers. Histopathological findings are calcification of small arteries and arterioles and infarction of subcutis and skin. The prognosis of calciphylaxis is poor due to an increased risk of systemic infection. METHODS: We report on a 50-year-old woman suffering from calciphylaxis. Initial treatments were not tolerated due to pain and therefore the patient was treated with maggot therapy and 800 mg/day of oral pentoxyfillin. RESULTS: Over a period of 6 months a complete remission of her skin lesions was achieved. CONCLUSION: Patients suffering from ulcers due to calciphylaxis may benefit from the use of maggot therapy, which cleanses ulcers and prevents systemic infection.

Administration, Oral↗

PubMed2001

Hyperbaric oxygen in the treatment of calciphylaxis: a case series.

BACKGROUND: Calciphylaxis, also referred to as calcific uraemic arteriolopathy, is a syndrome associated with end-stage renal disease (ESRD), and causes necrotic skin ulcers, often leading to a fatal outcome. Hyperbaric oxygen (HBO(2)) therapy has been used to enhance wound healing, but its role in the treatment of calciphylaxis is unclear. METHODS: We undertook a retrospective study of patients on renal replacement therapy with biopsy-proven calciphylaxis who were treated with HBO(2) between March 1997 and February 2000. RESULTS: Five patients were treated with HBO(2): three patients were on continuous ambulatory peritoneal dialysis (CAPD) and two were on chronic haemodialysis therapy. None of the patients had uncontrolled hyperparathyroidism and none underwent parathyroidectomy. The patients each received 25-35 treatments of HBO(2) at 2.5 atmospheres for 90 min per treatment. Two of these patients had complete resolution of extensive necrotic skin ulcers, with no adverse effects of HBO(2) therapy. Both had improvement in wound area transcutaneous oxygen pressure (P(tc)O(2)) with administration of 100% oxygen when measurements were taken at normobaric and hyperbaric pressures. In the other three patients receiving HBO(2), the skin lesions did not resolve. P(tc)O(2) was measured in two of these patients, neither of whom showed improvement with 100% oxygen administered at normobaric pressure. CONCLUSIONS: The data support a role for HBO(2) in the treatment of some patients with calciphylaxis, particularly as in the absence of uncontrolled secondary hyperparathyroidism there are few therapeutic options.

Calciphylaxis↗

PubMed1996

Calciphylaxis with histologic changes of pseudoxanthoma elasticum.

Calciphylaxis is a rare condition of widespread calcification of tissues and blood vessels with accompanying vascular thrombosis and ischemic necrosis. Most cases develop in association with hyperparathyroidism in patients with chronic renal failure. Pseudoxanthoma elasticum (PXE) is a hereditary condition of abnormal elastic tissue structure that leads to widespread abnormalities of the skin, retina, and visceral organs. Histologic changes of PXE have been observed as coincidental findings in several conditions such as following trauma to the skin manifest as isolated plaques often in scars. We observed histologic findings of PXE in a patient with chronic renal failure who developed fatal calciphylaxis. Complete evaluation failed to reveal evidence of systemic findings of PXE. Histologic changes of PXE may be seen in patients with calciphylaxis as a coincidental finding. Rapidly developing soft tissue calcification may lead to the expression of the characteristic histopathologic findings of PXE without evidence of classic clinical manifestations of PXE. Calciphylaxis should be added to the list of disorders that may lead to microscopic PXE-like changes.

Calciphylaxis↗

PubMedDate not supplied

Vacuum-assisted closure use in calciphylaxis.

Calciphylaxis-induced chronic wounds are difficult to heal. The value of vacuum-assisted closure (VAC) was assessed in two patients with calciphylaxis. Two middle-aged females with type 2 diabetes were transferred to the burn unit with a clinical diagnosis of necrotizing fasciitis, although the pathologic diagnosis was calciphylaxis. With extensive debridement, antibiotics, and meticulous wound care, one patient had progressive necrosis of her skin from 18 to 48% TBSA, whereas the other progressed from 5 to 10% TBSA only. The patient with the smaller chronic wound healed well and left the hospital at 72 days after admission. Although there was some success with the use of VAC, the patient with the extensive progressive wounds developed a fungal wound infection that did not respond to treatment; she died 78 days after initiation of burn center treatment. Some disadvantages to the usefulness would be extensive contiguous wounds and the lack of an intact skin surface. The VAC system was of value in healing wounds resulting from calciphylaxis.

Calciphylaxis↗

PubMed2004

Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy.

BACKGROUND: Calciphylaxis and metastatic calcification are known complications of chronic renal failure. Recently, a sclerosing condition of the skin termed nephrogenic fibrosing dermopathy (NFD) has been described in patients with renal disease, many of whom have undergone hemodialysis and/or renal transplantation. To our knowledge, the simultaneous occurrence of both conditions in the same patient, in the same lesion, has not been previously reported. CASE REPORT: We report the clinical, microscopic, and immunohistochemical features of two patients with chronic renal failure whose lesional skin biopsies showed both subcutaneous calcification and NFD. We consider the possible mechanisms that might explain the coexistence of these two disorders. RESULTS: Both patients presented with erythematous, indurated skin over the lower extremities. Purpuric, reticulated patches, necrosis, or ulceration were not observed. Microscopic examination showed the characteristic changes of NFD involving dermis and subcutaneous septa. In addition, biopsies of both individuals showed subcutaneous calcification, one in a diffuse distribution and the other involving the walls of subcutaneous vessels, as seen in calciphylaxis. Calcification was not suspected clinically in either case. CONCLUSIONS: Metastatic calcification or calciphylaxis and NFD can occur simultaneously in patients with chronic renal failure and may be found together in the same lesion. Because subcutaneous calcification may not be suspected clinically in these cases, and in view of the adverse outcomes frequently associated with calciphylaxis, we recommend deep incisional biopsy of patients presenting with the clinical features of NFD. Both the fibrosis and the calcification of chronic renal failure may be related to the activity of transforming growth factor-beta/Smad signaling cascades.

Adult↗

PubMed2003

Weight loss-induced calciphylaxis: potential role of matrix metalloproteinases.

Calciphylaxis is an uncommon and often devastating syndrome of calcification of small vessels, leading to tissue infarction. The mechanism of how calcium deposits on small vessels is unknown. Recently, metalloproteinase digestion of elastin has been shown to enhance deposition of calcium, suggesting a possible mechanism of calciphylaxis. We describe a case of a patient who developed calciphylaxis after rapid weight loss, but had normocalcemia and normal renal function. She was found to have high levels of matrix metalloproteinases, which may have chemically altered elastin, allowing deposition of calcium on small vessels. Inhibitors of matrix metalloproteinases may be useful in the prevention and treatment of calciphylaxis.

Adult↗

PubMedDate not supplied

Long-term intravenous sodium thiosulfate in the treatment of a patient with calciphylaxis.

Calciphylaxis is a disabling and life-threatening complication that primarily affects patients who are dialysis dependent. This disease entity is characterized by calcification, intimal hypertrophy, and thrombosis of small vessels, which results in necrotizing, nonhealing ulcers. The development of these lesions portends a grim prognosis, as they are often accompanied by severe and sometimes fatal infectious complications. Although several strategies aimed at treating and preventing this affliction have been reported in the literature, the outcome for most patients with calciphylaxis remains quite poor. We describe an anuric hemodialysis patient who developed severe calciphylaxis that proved refractory to conventional treatment. Following extensive debridement of several wounds, intravenous sodium thiosulfate was utilized as an adjunctive therapy four times a week. Within 6 months, excellent wound healing ensued as well as a dramatic improvement in the lesions that were not previously resected. Aside from occasional nausea, these treatments were well tolerated, despite the development of an anion gap metabolic acidosis. After 34 months of continued treatment the patient remains free from disease recurrence and has demonstrated no untoward effect of prolonged therapy. In an effort to delineate the pharmacokinetics of this drug in a hemodialysis patient, serum thiosulfate levels were obtained 15 minutes after infusion as well as before and after subsequent dialysis treatments. Consistent with prior studies in anuric canines, we found measurable quantities of the drug more than 50 hours after treatment, in addition to a markedly elevated half life of 478 minutes. However, given the lack of significant toxicity, as well as a dramatic clinical improvement, we feel that sodium thiosulfate may have an important adjunctive role in the treatment of calciphylaxis in dialysis patients.

Anuria↗

PubMedDate not supplied

Fatal childhood calciphylaxis in a 10-year-old and literature review.

Calciphylaxis is a rare but highly morbid disorder of vascular calcification and skin necrosis, affecting 1% to 4% of adults with end-stage renal disease. Only three affected children have previously been described. We report an unusual instance of fatal calciphylaxis involving a 10-year-old boy, in the setting of sarcoidosis-induced end-stage renal disease. A review of pediatric calciphylaxis cases suggests: (a) increased risk in boys with end-stage renal disease and secondary hyperparathyroidism; (b) frequent distal extremity and visceral organ involvement, (c) worse prognosis with acral necrosis, and (d) possible increased resistance to medical treatment compared to adult patients. Hyperesthetic pain is a common sentinel symptom, and soft tissue x-rays or xeroradiography or both may aid in the diagnosis of pediatric calciphylaxis. Parathyroidectomy in combination with supportive medical management may be the best treatment option in stopping the progression of disease in the pediatric population.

Calciphylaxis↗

PubMed1996

Severe calciphylaxis in a renal patient on long-term oral anticoagulant therapy.

The pathogenesis of calciphylaxis, a potentially life-threatening condition, is not well understood. Several factors such as end-stage renal disease (azotemia), hyperparathyroidism, hyperphosphatemia, hypercalcemia, a high calcium-phosphate product, and the use of steroids and cytotoxic drugs after kidney transplantation are believed to interact in calciphylaxis. Recently, hypercoagulability due to functional protein C deficiency has been suggested to play a pathogenic role in this condition. Here, we present a renal transplant patient, with secondary hyperparathyroidism and on long-term oral anticoagulant therapy, who developed calciphylaxis with severe skin necrosis of her legs. The patient's condition improved dramatically after total parathyroidectomy. Hypercoagulability, therefore, does not appear to have played a significant role in this case of calciphylaxis.

Administration, Oral↗

PubMed2000

Calciphylaxis: seven new cases.

BACKGROUND: Calciphylaxis is a rare condition occurring most frequently in patients with end-stage renal disease (ESRD). It is characterized by vascular calcifications with a large variety of skin lesions. Even though this entity was first reported almost 50 years ago, the pathogenesis is still not well understood. OBJECTIVE: Having retrieved seven new cases from a single tertiary care hospital, the disease occurs probably more frequently than reported until now. The potential mechanism of action in this disease is discussed, particularly the hypercoagulability state. We also review potential treatments described in the literature. METHODS: Seven patients with calciphylaxis that occurred at the Hôtel-Dieu hospital between 1992 and 1998 were identified and their case histories reviewed and analyzed. CONCLUSION: Although hyperparathyroidism and imbalance of calcium-phosphorus homeostasis are paramount for calciphylaxis to occur, other mechanisms must be involved because the disease manifests itself in only a minority of ESRD patients. As the majority were under anticoagulation therapy and as we found abnormalities of the coagulation pathway in one patient, we suggest emphasizing these phenomena in the future. Along with evaluation of putative risk factors (abnormalities of the calcium:phosphate axis, diabetes), a detailed evaluation of the coagulation system should be done in every patient with calciphylaxis until more data are available.

Adult↗

PubMed1998

Calciphylaxis: a cause of necrotic ulcers in renal failure.

BACKGROUND: Calciphylaxis is a rare and life-threatening condition occurring in patients with end-stage renal disease on dialysis. The diagnosis is frequently delayed or missed and therapy is often unsuccessful. OBJECTIVE: The clinical and pathological components of calciphylaxis are emphasized in a patient that may facilitate early diagnosis and initiation of therapy. RESULTS: Early recognition of the clinical signs and symptoms of calciphylaxis is crucial. The tissue diagnosis requires large elliptical biopsies because histological findings are segmental and often missed. CONCLUSION: Early diagnosis and intervention in calciphylaxis is critical. Parathyroidectomy should be considered in all cases.

Biopsy↗

PubMed2005

Development of calciphylaxis after long-term steroid and methotroxate use in a patient with rheumatoid arthritis.

Calciphylaxis may be considered a small vessel vasculopathy which is generaly associated with end-stage renal disease and hyperparathyroidism. The precise pathogenesis of the disease is not known. It needs sensitizers and challengers to occur. Steroids and immunosuppressive drugs including methotrexate are among those challenger agents. Calciphylaxis in collagen vascular diseases is rare. Only one case in rheumatoid arthritis was recently reported. Here we describe a case of calciphylaxis associated with active rheumatoid arthritis. This patient had active disease despite treatment of steroids and methotrexate for a long time. She died shortly after the diagnosis of calciphylaxis due to sepsis.

Adult↗

PubMed2002

[The calciphylaxis syndrome as a complication of chronic kidney failure. 6 case reports and literature review].

Calciphylaxis is a rate complication of unknown pathogenesis in patients with end stage renal disease. It is characterized by calcification of tunica media of small arteries associated with intimal fibrosis and thrombus formation which leads to the development of skin and subcutaneous tissue necrosis. Superinfection of skin lesions is a common consequence of this syndrome which may lead to the sepsis. The prognosis of this condition in serious. We performed a retrospective study of 6 subjects (4 men and 2 women) in the age of 35 to 59 years. We followed the parameters of calcium-phosphate metabolism, presence of calciphylaxis risk factors and the effect of parathyreoidectomy. Five patients were on hemodialysis, one had a kidney transplant. Skin and subcutaneous tissue necrosis were present in all subjects. The serum levels of parathormone were either high, normal or low, levels of calcium were normal or slightly elevated and phosphate levels were high or normal. Calcium was substituted before calciphylaxis development in 5 patients, calcitriole in 3 of therm. Five patients underwent parathyroidectomy. Three patients died (all of sepsis), one patient had the lower into amputation for infected lesions and the remaining two achieved regression. Our findings do support the hypothesis that calcium and calcitriole administration participates in development of calciphylaxis. Fatal prognosis of the once infections skin lesions was also proved.

Adult↗

PubMed2003

Calciphylaxis: a case study with differential diagnosis.

Calciphylaxis is a relatively rare, but horribly disfiguring, skin condition that is most often associated with end-stage renal disease and long-term dialysis. Unfortunately, calciphylaxis-related morbidity and mortality are significant. The case study presented demonstrates many of the findings associated with the typical calciphylaxis patient; end-stage renal disease and an extensive, painful ulcer. The complexity of the patient s history and medical/surgical interventions, especially medication therapy with coumadin and heparin, complicated initial diagnostic processes. Close scrutiny of multiple physical assessment findings, historical factors, and test results was required for correct diagnosis. Crucial components of differential diagnosis of calciphylaxis versus coumadin-induced skin necrosis or heparin-induced thrombocytopenia necrosis include: patient history and characteristics, clinical presentation, and diagnostic test results.

Amputation, Surgical↗

PubMed2003

Protracted calciphylaxis, Part I.

Calciphylaxis is a serious and often lethal condition that mostly affects patients with renal disease. Patients with calciphylaxis typically have variable degrees of cutaneous necrosis on initial presentation. An unusual, protracted course of calciphylaxis without cutaneous ulcerations has been encountered in the case of a 46-year-old woman. Thirteen additional cases with similar presentation will be discussed in part II of this article. Calciphylaxis may include several clinical presentations, ranging from an acute, rapidly fatal course to an indolent, more benign variant.

Biopsy↗

PubMed2004

Mysterious calciphylaxis: wounds with eschar--to debride or not to debride?

Calciphylaxis is a confusing disease process that affects people with end-stage renal disease. The prognosis of this increasingly common condition is poor and mortality rates range from 60% to 80% related to wound infection, sepsis, and organ failure. Its presenting sign is skin necrosis related to calcification of the arteriole microvasculature. The disease is painful and debilitating, particularly due to the necrotic wounds. Aggressive wound care to prevent infection is vital when eschar does not protect the wound and drainage is present, but debridement is contraindicated for wounds covered with dry, noninfected eschars. The decision to debride is based on the patient's total clinical picture. Patients with calciphylaxis have poor healing potential due to ischemia and comorbidity factors such as diabetes mellitus, peripheral vascular disease, and obesity. The goal of care is prevention of infection and pain management. Some of the sensitizers and challengers responsible for the chemical imbalance leading to the arteriole calcification, as well as risk factors and clinical manifestations of calciphylaxis, are reviewed. A discussion of treatment focuses on wound care of stable necrotic ulcers and a case report illustrating the progression of calciphylaxis is presented.

Calciphylaxis↗

PubMed1991

Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy.

Calciphylaxis is a rare, severe complication of secondary hyperparathyroidism. Patients present with painful, violaceous, mottled skin lesions of the upper and lower extremities, which become necrotic and produce nonhealing ulcers. Gangrene of fingers and toes frequently requires amputation, produces nonhealing wounds, and can lead to sepsis and death. We reviewed the clinical course of five patients with calciphylaxis treated in our institution. The three men and two women (aged 47 to 72 years) had secondary hyperparathyroidism from chronic renal failure. All patients had severe pruritus, painful ulcers, and severe hyperphosphatemia with elevated serum calcium-phosphate product (greater than 12 mmol2/L2), but the serum parathyroid hormone levels were only moderately elevated. Most patients had medical calcification of medium and small blood vessels, and some had soft-tissue calcification visible on roentgenography. Treatment consisted of local wound care, antibiotics, phosphate-binding agents, and parathyroidectomy. Two patients died of uncontrollable sepsis. The three survivors had dramatic improvement of pain and ulcers after parathyroidectomy. Calciphylaxis is a limb- and life-threatening complication of secondary hyperparathyroidism. Diagnosis can be made by recognizing the characteristic painful skin lesions, ulcers, and gangrene of the digits, and patients should be treated with subtotal parathyroidectomy.

Aged↗

PubMed2000

[Calciphylaxis: ischemic tissue necrosis in chronic renal failure. Case report and review of the literature].

Calciphylaxis is a rare but potentially life-threatening complication in chronic renal failure. It is characterized by ischemic tissue necrosis primarily of the skin. The typical histopathologic finding is microvascular calcification with endovascular fibrosis. Patients typically present with violaceous, mottled and painful lesions which tend to progress to non-healing ulcers and necrosis. Most frequently the lower extremities are involved in a symmetric fashion but the trunk may also be affected. Sepsis from superinfection of the lesions accounts for the high mortality of this disease which is of importance for dermatologists and nephrologists alike. 61-year-old female patient developed lesions of calciphylaxis on both calves two years after beginning hemodialysis to treat renal failure due to diabetic glomerulosclerosis. We discuss aspects of the pathogenesis of calciphylaxis, as well as diagnosis, treatment and means of prevention, and review the current literature.

Biopsy↗