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Results for “CALCIPHYLAXIS”

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PubMed2002

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.

Calciphylaxis↗

PubMed2006

Calciphylaxis associated with alcoholic cirrhosis.

Calciphylaxis is an uncommon disease characterized by calcification of dermal vessels that determines skin necrosis. Calciphylaxis has been almost exclusively reported in association with renal failure and altered phosphor-calcium metabolism. Only a few cases have been described in hyperparathyroidism, malignancies, and, recently, cirrhosis. We report a patient that developed calciphylaxis related to end-stage alcoholic cirrhosis, without any alteration in the phosphocalcic and parathyroid hormone metabolisms. Possible contributing factors were repeated albumin infusions and low levels of protein C and S.

Biopsy↗

PubMed1998

Calciphylaxis: pathogenesis and therapy.

BACKGROUND: Calciphylaxis is a rare, painful and debilitating disease in which calcification of the skin and subcutaneous tissues or of internal organs can lead to skin necrosis, discolouration, and other skin lesions. The typical patient has end-stage renal disease (ESRD) and hyperparathyroidism. Selye originally characterized this syndrome in rats and distinguished it from other syndromes of abnormal calcification by the following sequence: sensitizers, latent period, and challengers. There has been recent debate regarding misdiagnosis and failure to differentiate this category of patients even in clinical studies. OBJECTIVE: In this article the clinical manifestations of calciphylaxis are described; the importance of distinguishing this condition from other calcification syndromes is explained; risk factors, sensitizers, and challenges are reviewed; treatments of choice are discussed; and the merits of parathyroidectomy are evaluated. CONCLUSION: It is important to consider calciphylaxis in a differential diagnosis of calcification syndromes and in treating patients with ESRD and hyperparathyroidism, because early diagnosis and treatment can interrupt the progression of the disease process; the disease is painful and debilitating; and inappropriate treatment such as corticosteroid administration may aggravate the condition. Skin biopsy is an important diagnostic tool when the condition is suspected. Parathyroidectomy may be justified because the untreated disease itself has significant rates of morbidity and mortality and because this treatment occasionally leads to dramatic clinical improvement.

Animals↗

PubMed1998

Calciphylaxis mimicking dermatomyositis: ischemic myopathy complicating renal failure.

BACKGROUND: Among the complications of chronic renal failure is a syndrome of medial calcification of small- to medium-sized arteries associated with ischemic necrosis of the skin and other organ systems, leading to gangrene and a poor prognosis. The syndrome has been reviewed in the renal, dermatologic, and surgical literature under the term calciphylaxis, which describes a postulated pathogenetic mechanism whereby sensitization to an endogenous or exogenous substance (such as parathyroid hormone) predisposes to calcium deposition after exposure to a challenging agent. Myopathy has rarely been reported as the presenting feature, and the syndrome has not been discussed in the neurologic literature. METHODS: We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myopathic complaints and signs suggesting dermatomyositis. We also discuss possible disease mechanisms and treatment. CONCLUSIONS: Because early treatment (including aggressively lowering the calcium and phosphate levels and parathyroidectomy) may improve the outcome, early recognition of the syndrome of calciphylaxis is essential.

Adult↗

PubMed2004

Anesthetic management of patients with severe peripheral ischemia due to calciphylaxis.

Calciphylaxis is a small-vessel disease associated with renal failure. Here, we report the management of a 43-yr-old man with calciphylaxis who received left lower leg amputation with prostaglandin E(1) (PGE(1)) under monitoring by laser Doppler blood flowmetry in the left second and third fingers. Anesthesia was induced with midazolam, fentanyl, and vecuronium and was maintained with oxygen, nitrous oxide, and sevoflurane. The peripheral blood flow varied and decreased gradually; therefore, we added PGE(1) 20 ng. kg(-1). min(-1), which increased blood flow of the tissues. Three weeks after the operation, we again anesthetized the patient. We maintained the blood flow with PGE(1) throughout anesthesia. Monitoring by laser Doppler blood flowmetry and PGE(1) 20 ng. kg(-1). min(-1) could be useful for patients with impaired peripheral circulation, as in calciphylaxis.

Adult↗

PubMed2006

Long-term outcomes in patients with calciphylaxis from hyperparathyroidism.

BACKGROUND: Calciphylaxis is a rare condition associated with chronic renal failure and entails a very poor prognosis. Pathogenesis is poorly understood but involves abnormalities in calcium and phosphorus metabolism that lead to vascular and extravascular calcification. Patients present with painful ulcerating plaques that progress to gangrenous wounds. Parathyroidectomy has been advanced as a life-saving intervention in these patients, but long-term results with wound healing and survival after parathyroidectomy are not well described. METHODS: Between January 1987 and October 2003, 15 patients with biopsy-confirmed calciphylaxis were treated at the University of Wisconsin. Of these 15 patients, 9 were treated with medical therapy (bisphosphonates and phosphate binders), whereas 6 underwent parathyroidectomy. The medical records were reviewed, and patients or relatives were interviewed. Survival was determined by Kaplan-Meier analysis. RESULTS: Four patients underwent subtotal parathyroidectomy, and two patients underwent total parathyroidectomy. All had reductions in the intact parathyroid hormone level (mean +/- SD, 25.2 +/- 4.5 pg/mL). Whereas all six patients treated with parathyroidectomy had partial/complete wound healing, only two of nine in the medical group had any improvements in the skin lesions (P = .006). With up to 80 months of follow-up, patients who underwent parathyroidectomy had a longer median survival compared with those who did not have surgery (39 vs. 3 months; P = .017). CONCLUSIONS: On the basis of our long-term follow-up of this patient population, subtotal or total parathyroidectomy was associated with long-term survival and was more likely to promote healing if performed earlier in the course of disease. Therefore, patients with calciphylaxis from secondary hyperparathyroidism should be referred promptly for potential parathyroidectomy.

Adult↗

PubMed1996

Calciphylaxis in chronic renal failure.

Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. Typically, extensive microvascular calcification and occlusion/thrombosis leads to violaceous skin lesions, which progress to nonhealing ulcers and sepsis. Secondary infection of skin lesions is common, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis. Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene. The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory. Patients who undergo parathyroidectomy have a tendency to improve, but the prognosis for the disease is poor and mortality remains high.

Adult↗

PubMed2003

A strategy for the treatment of calcific uremic arteriolopathy (calciphylaxis) employing a combination of therapies.

Calcific uremic arteriolopathy (calciphylaxis) is one of the more devastating complications that can develop in patients with chronic renal failure. This disorder is associated with calcium-phosphorus deposition in the subcutaneous arterial vessels and presents as a progressive ischemic necrosis of the skin resulting in large subcutaneous ulcerations with eschar formation. Mortality rates are substantially greater in chronic renal failure patients with calciphylaxis, and the major cause of death is infection and sepsis. We have developed a treatment strategy that employs a combination of therapies, which is based on reducing the known risk factors for the development of calciphylaxis as well as utilization of a number of treatment modalities that have been proven successful in the treatment of this disorder.

Calciphylaxis↗

PubMed2000

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.

Adult↗

PubMed2001

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.

Aged↗

PubMed2002

Calciphylaxis: what nurses need to know.

Calciphylaxis is a rare and potentially fatal complication of end stage renal disease (ESRD) and secondary hyperparathyroidism associated with abnormal calcium metabolism. Painful purple skin lesions are often the presenting sign with digital ischemia, ulcerations, and necrosis being the characteristic features of the disease. Skin or incisional biopsy showing widespread calcifications and fibrinous thrombi without inflammation will confirm the diagnosis. Early recognition and prompt treatment of calciphylaxis is vital for symptom relief, ulcer healing, and prevention of sepsis and death. This article will educate nurses to recognize this disease in hope of early treatment. The article includes information about disease history, pathogenesis, etiology, clinical manifestations, diagnosis, prevention, and current treatment, and, in addition, presents case study of a patient with calciphylaxis.

Calciphylaxis↗

PubMed2002

Calciphylaxis: case report and literature review.

Tissue calcification is a well-recognized common metabolic disease, but calciphylaxis still remains an enigmatic rarity. The latter may be induced experimentally and acquired naturally in human diseases. Although many chronic azotemic or end stage renal disease patients (ESRD) with hyperparathyroidism (HPT) are at risk of calciphylaxis not all of them do develop the disease, even non-renal, patients may also develop this disease. Out of a total of about 2000 hemodialysis, 15,000 dermatology and 26,000 medical patients seen over a three year period in a busy Saudi Arabian tertiary medical centre, we report a sentinel nephrology patient with sudden excruciatingly painful cutaneous calciphylaxis that necessitated acute dermatology emergency consultation, and present a review of the medical literature. In order to institute appropriate total quality management of this life-threatening, rare disease, it is advisable that a high index of suspicion should be entertained by dermatologists, general physicians, nephrologists, and pulmonologists in an appropriate clinical scenario.

Acute Disease↗

PubMed2003

Subtotal parathyroidectomy: a possible treatment for calciphylaxis.

Calciphylaxis is a rare disorder in patients with chronic renal failure that is characterized by ischemic necrotic skin lesions. The prognosis is grave and mortality is high (80%). The precise mechanism of calciphylaxis is still unknown, but in addition to chronic renal failure, elevated parathyroid hormone levels appear to play a role. The role of parathyroidectomy in treating affected patients is questionable. In this article, we describe the case of a patient with chronic renal failure who developed rapidly progressive subcutaneous calcifications and ulcerations in the lower extremities. These lesions regressed following subtotal parathyroidectomy. We also review the literature on calciphylaxis, with a focus on treatment options.

Biopsy, Needle↗

PubMed2003

Protracted calciphylaxis, Part II.

An unusual protracted course of calciphylaxis without cutaneous ulcerations has been encountered in 14 cases. Calciphylaxis may include several clinical presentations ranging from an acute, rapidly fatal course to an indolent, more benign variant. Calciphylaxis should be included in the differential diagnosis for patients with kidney disease presenting with a clinical picture of panniculitis. Patients with renal disease with a characteristic clinical presentation with or without cutaneous ulcerations should undergo an urgent biopsy to confirm the diagnosis.

Adult↗

PubMed2004

Calciphylaxis involving both the upper and lower extremities.

Calciphylaxis is an uncommon complication of end stage renal disease (ESRD) and secondary hyperparathyroidism. It characterized by cutaneous necrosis with mural calcifications and thrombosis in the small vessels of dermis. It is important to diagnose and treat, because of mortality rate from calciphylaxis is very high. We present the case of a patient with ESRD and type II diabetes mellitus developing calciphylaxis of the both upper and lower extremities had normal corrected calcium-phosphate product level. After amputation, necrosis was showed rapid progression resulting in death in one month.

Calciphylaxis↗

PubMed2005

A case of calciphylaxis and chronic myelomonocytic leukemia.

A 70-year-old woman presented for evaluation of symmetric necrotic ulcers of the lower extremities. Biopsy results revealed changes consistent with calciphylaxis. The predisposing factors in this patient included calcium supplementation, obesity, female gender, viscous blood, renal failure, and diabetes mellitus. To our knowledge, this is the first report of calciphylaxis occurring in the setting of chronic myelomonocytic leukemia. We discuss the history, clinical presentation, diagnosis, and treatment of calciphylaxis.

Aged↗

PubMed2005

[Sterile maggots as adjuvant procedure for local treatment in a patient with proximal calciphylaxis].

Proximal calciphylaxis with skin ulcerations has a very poor survival and infection is the main cause of the high mortality rate. We present the case of a diabetic obese hemodialysis woman diagnosed of severe calciphylaxis with extensive ulcers in the abdomen and left thigh. After a first ineffective debridement attempt, systemic medical treatment was associated with daily wet cures with enzymatic ointments and maggot therapy resulting in spectacular granulation and healing of these ulcers. We want to underline the great properties of sterile maggots to selectively dissolve necrotic tissue, to disinfect the wound and to stimulate healing. Thus, sterile-maggot debridement may constitute a very good adjuvant treatment even in proximal ulcers of calciphylaxis, preventing systemic infection.

Animals↗

PubMed1989

[Clinical aspects of metabolic nephropathies, interstitial nephritis and urolithiasis in calciphylaxis].

The problem of abacterial nephropathies associated with impaired calcium metabolism is discussed from the standpoint of membranous pathology. The data are presented on the similarity of the calciphylaxis pathogenesis described for the first time by H. Selye to calcium nephrolithiasis. The disease is viewed as the result of pronounced modification of the phospholipid layer of cell membranes under the influence of peroxidation, phospholipase activity and other processes induced by calcium-regulating hormones (parathyroid hormone, D3 and calcitonin). It is assumed that the clinical manifestations of renal pathology during calciphylaxis depend on the hereditary and environmental factors that determine the gravity of membranous pathology of renal cells. Recommendations as to the treatment and prevention of renal pathology during calciphylaxis based on the results of the authors' own experience are given.

Calcinosis↗