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PubMed1995

[Cutaneous calciphylaxis in chronic renal failure].

A case of a 53-year-old chronically uraemic female patient who suddenly developed widespread cutaneous necrosis on both thighs is presented. On the basis of the typical histopathological features a diagnosis of calciphylaxis was made and supported by elevated serum parathyroid hormone levels. Calciphylaxis is a rare and serious disease, mostly seen in uraemic patients subjected to chronic haemodialysis. Typical features are calcifying panniculitis and thrombosis of small to medium-sized blood vessels followed by necrosis of the skin. The poor prognosis is due to systemic calcifications of visceral organs. When acute cutaneous necrosis of the skin is encountered in patients with chronic uraemia calciphylaxis should always be considered as a possible cause in the differential diagnosis. Determination of the serum parathyroid hormone level is recommended.

Biopsy↗
PubMed1999

[Disseminated ischemic necrosis and livedo racemosa in a chronic dialysis patient with calciphylaxis].

Calciphylaxis occurred in a 40-year old female patient with end-stage renal failure. The patient developed livedo racemosa ("livedo reticularis") with painful skin necrosis and ulcers involving multiple areas of the hip and legs after 22 years of hemodialysis. X-ray-examinations revealed calcinosis of peripheral arteries, especially of the pelvis, thigh and hands, while histological examinations showed a fibrosis and calcinosis of small subcutaneous arteries. A generalized cutaneous microangiopathy could be demonstrated by transcutaneous oxygen pressure measurements. Laboratory data showed a moderate secondary hyperparathyroidism with mild elevation of calcium-phosphate product. In addition to the hemodialysis an attempt was made to improve the microcirculation by vasoactive drugs. The clinical course was characterized by slow healing of the ulcers and occurrence of new areas of cutaneous necrosis. Calciphylaxis is a rare late complication in patients with advanced, often end-stage renal failure. It has characteristic histopathological features and is frequently, but not always, associated with a disturbed calcium and phosphorus metabolism and mildly elevated levels of parathyroid hormone. Calciphylaxis is classified as a special type of metastatic calcinosis.

Adult↗
PubMed2005

[Calciphylaxis: a rare differential diagnosis of systemic vasculitis].

INTRODUCTION: Calciphylaxis is a rare phenomenon of medium- and small-vessel calcifications leading to cutaneous necrosis mimicking vasculitis. CASE REPORT: A 75 year-old-woman with chronic renal insufficiency was admitted for extensive cutaneous necrosis of the limb. Diagnosis of vasculitis was made, but inspite of corticosteroid therapy, the condition of the patient was worsening. After cutaneous biopsy, the diagnosis of calciphylaxis was established. CONCLUSION: Calciphylaxis must be suspected in cases of cutaneous necrosis occurring in a patient with chronic renal failure. Treatment requires rapid normalization of phosphocalcic balance. It is a condition with high mortality.

Aged↗
PubMed2002

Penile calciphylaxis.

Calciphylaxis is an uncommon condition usually seen in patients with end-stage renal disease. The typical features include violaceous skin lesions overlying painful, indurated, subcutaneous nodules. Necrosis and nonhealing ulcers, with secondary gangrene, sepsis, and death frequently follow. The outpatient hemodialysis population has a reported calciphylaxis prevalence of 1% to 4.1%; however, published studies contain only a few case reports of penile calciphylaxis. The urologic presentation consists primarily of penile gangrene. A description of our patient, the underlying pathologic features, a review of the relevant published studies, and the possible predisposing conditions are included.

Calciphylaxis↗
PubMed1999

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.

Adult↗
PubMed1990

Calciphylaxis induced by iron-citrate sorbitol.

The author describes an experimental model of calciphylaxis induced by iron citrate sorbitol in young dogs. The lesions became apparent in form of nodular white and hard papules 0.1-0.5 cm in diameter 5-7 d after subcutaneous administration of iron. Microscopically a granulomatous reaction of the dermis and hypodermis are visible and also some associated modifications, as adipose cysts and fibrillar densification. From histochemical points of view a highly positive von Kossa reaction is observed on the adipocyte's membrane, on collagen fibers or as a compact extensive material. The significance of the morphohistochemical appearances is discussed as a dynamic process which leads to calciphylaxis of the skin and then to a granulomatous reaction of foreign body type. We suggest that this experimental model of calciphylaxis could serve as an explanation for some calcifications observed in human pathology.

Animals↗
PubMed2000

Calcified subcutaneous arterioles with infarcts of the subcutis and skin ("calciphylaxis") in chronic renal failure.

Patients with chronic renal failure (CRF) are at increased risk for pathological calcifications because of increased serum calcium-phosphorus products. A minority, including those undergoing dialysis, develop a syndrome of deep skin ulcerations in association with calcification of subcutaneous arterioles. The body distribution of the skin lesions may be proximal (central), distal (peripheral), or both. Since 1968, this syndrome has been called "calciphylaxis" in the belief that it is the human analogue of Selye's experimental models of tissue calcification. Our review emphasizes that this syndrome comprises two separate processes not found in calciphylaxis: calcification of subcutaneous arterioles and infarctions of subcutaneous adipose tissue (panniculus adiposus) and skin. The infarctions are acute and clinically dramatic, whereas the calcific arteriolopathy is preexistent, having developed slowly, sometimes over years, and silently. Separating these two processes facilitates analyses of pathogenetic factors, such as those that target subcutaneous arterioles for calcification and those that interfere with blood flow through the calcified arterioles, sufficient in some patients to cause the infarctions, and of why obesity in CRF is a syndrome risk factor. This approach further helps to provide a much needed standardized definition of the syndrome, thereby facilitating comparisons of the results of such treatments as parathyroidectomy, anticoagulants, and phosphate binders. Finally, the separation shows why the application of such terms as calciphylaxis and calcifying panniculitis to this syndrome is inappropriate.

Animals↗
PubMed1992

Calciphylaxis in a hemodialysis patient: appearance after parathyroidectomy during a psoriatic flare.

Chronic renal failure patients are prone to soft tissue calcifications. A phenomenon of acute ischemic skin necrosis and dermohypodermic arteriolar medial calcification has been described recently in patients with chronic renal failure and secondary hyperparathyroidism (HPT). This phenomenon, termed calciphylaxis, occurs in response to certain factors, the most important of which appears to be an elevated blood calcium-phosphate product. Accordingly, parathyroidectomy in addition to normalization of calcium-phosphate product has been proposed as the only effective therapeutic approach for this condition. We describe a case of chronic renal failure with severe secondary HPT in which the patient developed calciphylaxis 4 days after the appearance of a psoriatic flare. Four months before, a subtotal parathyroidectomy was performed for severe HPT and at the time the ulcerations appeared, blood calcium-phosphate product was correct. Etiological and physiopathological aspects of calciphylaxis are discussed.

Calciphylaxis↗
PubMed1999

Calciphylaxis precipitated by ultraviolet light in a patient with end-stage renal disease secondary to systemic lupus erythematosus.

Calciphylaxis is a rare and severe calcification syndrome described mainly in patients with end-stage renal disease (ESRD) undergoing dialysis or with a renal transplant. This life-threatening condition is characterized by the abrupt onset of painful ischemic skin ulcers and necrosis. Secondary local and systemic infection may supervene and, without timely and appropriate interventions, calciphylaxis may be fatal. A precipitant or challenging agent is believed to be necessary to initiate the process. We describe a case of a woman with ESRD receiving continuous ambulatory peritoneal dialysis who developed calciphylaxis in the setting of severe hyperparathyroidism after receiving UV photoradiation therapy. We suggest that the UV light served as the challenging agent in the precipitation of this devastating condition.

Adult↗
PubMedDate not supplied

Calciphylaxis treated with neurolytic lumbar sympathetic block: case report and review of the literature.

BACKGROUND AND OBJECTIVE: Calciphylaxis is a painful complication of end-stage renal disease and secondary hyperparathyroidism. Calcification most commonly affects skin and soft tissue of the lower extremities resulting in excruciatingly painful skin ulcers. Treatment involves correction of hypercalcemia and hyperphosphatemia, parathyroidectomy, and supportive measures. METHODS: The literature and the merits of neurolytic lumbar sympathetic blockade (LSB) for the treatment of pain associated with calciphylaxis are reviewed. CONCLUSIONS: The neurolytic LSB provided pain relief and is a treatment modality to be considered in managing the pain associated with calciphylaxis.

Autonomic Nerve Block↗
PubMed2005

[Calciphylaxis in terminal renal failure as a rare manifestation of leg ulcers].

Calciphylaxis is a potentially life-threatening disease that occurs up to 4% of patients with chronic terminal renal failure and secondary hyperparathyroidism. Clinical symptoms are painful skin erythema and ischemic necrosis as a result of calcification of the small and medium-sized arteries of the subcutaneous tissues. The mortality of the disease is as high as 87% mainly due to sepsis from superinfection. We report on a 59-year-old patient with chronic renal failure and normal calcium-phosphate-product with leg ulcers as a rare manifestation of a calciphylaxis. In spite of an early parathyroidectomy and a temporary complete wound-healing the death because of calciphylaxis could not be prevented.

Calciphylaxis↗
PubMed2000

Calciphylaxis in the absence of renal failure.

We report a patient with severe bilateral leg ulceration that was resistant to treatment. A biopsy confirmed the cause as calciphylaxis. Calciphylaxis refers to a syndrome of calcium deposition in the small and intermediate dermal vasculature which can lead to epidermal ischaemia, ulceration and necrosis. Most cases occur in those with chronic renal failure and secondary hyperparathyroidism. We describe the rare presentation of calciphylaxis in a patient with normal renal function and primary hyperparathyroidism who had many classical features. Unfortunately she developed gangrene, sepsis and died.

Aged↗
PubMed1998

Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease.

Cryofibrinogenemia is a disorder characterized by cryoprecipitation with variable clinical presentation that was first described by Korst and Kratochvil in 1955. Cryofibrinogen is a cold insoluble complex of fibrin, fibrinogen, and fibrin split products with albumin, cold insoluble globulin, factor VIII, and plasma proteins. Cryofibrinogenemia is associated with metastatic malignancies, collagen vascular diseases, and thromboembolic disorders and may be clinically asymptomatic or present with thromboembolic phenomena of skin and viscera. The pathogenesis of cryofibrinogenemia is unknown. It may be caused by the inhibition of fibrinolysis, leading to an accumulation of cryofibrinogen. Treatment of cryofibrinogenemia may include Stanozolol, plasmapheresis, and fibrinolytics. Cryofibrinogenemia simulates calciphylaxis clinicopathologically, because both may present with skin necrosis. Calciphylaxis has been reported in end-stage renal disease, but we report the first case of cryofibrinogen in a chronic dialysis patient. We suggest that in the appropriate clinical setting, cryofibrinogenemia should be considered in the differential diagnosis of calciphylaxis, and serum cryofibrinogen levels should be measured in end-stage renal disease patients presenting with skin necrosis.

Arterioles↗
PubMed2000

Is calciphylaxis best treated surgically or medically?

BACKGROUND: Calciphylaxis is a rare, painful, life-threatening problem of cutaneous necrosis and refractory healing in patients with uremia and secondary hyperparathyroidism. The pathogenesis involves abnormalities in calcium and phosphorus metabolism and acute deposition of calcium in tissues. METHOD: The clinical course of 16 patients who were diagnosed with calciphylaxis at our institution from 1994 through 1998 was reviewed. RESULTS: Fourteen female patients and 2 male patients had chronic renal disease, secondary hyperparathyroidism, and characteristic skin necrosis (mean age, 56 years; range, 39-70 years). All patients underwent intensive medical therapy, including ongoing hemodialysis (n = 16 patients), parathyroidectomy (n = 7 patients), and debridement of cutaneous lesions (n = 8 patients). Mean serum values in surgical and nonsurgical patients were significantly different for phosphorus, calcium-phosphorus product, and parathormone levels. Median survival was 9.4 months; 15 patients (93%) have died. The median survival time for parathyroidectomy versus nonparathyroidectomy was 14.8 and 6.3 months (P =.22), for skin debridement versus nondebridement was 14.1 and 6.1 months (P =.08), and for diabetic versus nondiabetic patients was 6.5 and 13.9 months (P =.11). CONCLUSIONS: Calciphylaxis has a female preponderance, with a dismal prognosis. A multidisciplinary approach that uses frequent hemodialysis to normalize calcium and phosphorus levels and local debridement of skin lesions seems prudent. Parathyroidectomy cannot be recommended routinely in all patients, unless severe hyperparathyroidism mandates intervention.

Adult↗
PubMed2004

Successful treatment of severe calciphylaxis in a hemodialysis patient using low-calcium dialysate and medical parathyroidectomy: case report and literature review.

This investigation presents a case report of the successful treatment of severe calciphylaxis using zero- and low-calcium dialysate for hemodialysis, repeated wound debridement and skin grafting and medical parathyroidectomy with local injection of parathyroid glands with alcohol. To the authors' knowledge, this is the first case report of the use of medical parathyroidectomy and hemodialysis with low calcium dialysate to treat calciphylaxis in a dialysis patient. Finally, relevant English language articles on calciphylaxis from Medline are reviewed.

Adult↗
PubMed1996

Calciphylaxis mimicking skin lesions of connective tissue diseases.

Calciphylaxis is a rare condition found in patients with end-stage renal disease and secondary hyperparathyroidism. We describe two cases of prominent cutaneous manifestations of calciphylaxis in which skin lesions were refractory to topical treatments. We also include a brief description of calciphylaxis.

Adult↗
PubMed2004

Nanobacteria-caused mitral valve calciphylaxis in a man with diabetic renal failure.

We have found that nanobacteria, recently discovered Gram-negative atypical bacteria, can cause local calciphylaxis on the mitral valve in a setting of high-calcium X phosphorous product in the blood. We present the case of a 33-year-old man with diabetic renal failure on continuous ambulatory peritoneal dialysis who died as a result of multiple brain infarcts due to embolizations from mitral valve vegetations. Systemic calciphylaxis was not present. Spectrometric analysis of the mitral valve vegetations showed that they were composed of calcium phosphate, carbonate apatite form, and fibrin. The electron microscopy of the thrombotic vegetation demonstrated nanobacterium as a nidus for carbonate apatite formation. Investigation for the presence of nanobacteria in the multiple organs involved in systemic calciphylaxis may be of help in elucidating the pathogenesis of this frequently fatal disorder.

Adult↗