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Calciphylaxis is a rare condition characterized by skin ulceration and necrosis as a result of vascular calcification of the small and medium blood vessels of skin and subcutaneous tissues. It mainly occurs in patients with advanced chronic kidney disease and sometimes leads to complications with a fatal outcome. In this report, we describe the case of a 67-year-old male patient with end stage renal disease presenting painful skin ulcers on his lower limbs. The lesions had progressively grown and were associated to severe pain and decreased quality of life. The ulcers did not respond to conventional treatments and the patient underwent skin biopsy of these lesions obtaining anatomopathological findings compatible with calciphylaxis. In this report, we present an innovative treatment for skin ulcers secondary to calciphylaxis using cryopreserved amniotic membrane (AM) as a dressing in order to promote epithelialization of the wounds. After four applications, healing of the main ulcer and reduction in pain was achieved. In summary, applying cryopreserved AM probed to be a promising strategy to reduce pain and to enhance epithelialization and healing of chronic non-responsive ulcers in calciphylaxis., (© 2021 Wiley Periodicals LLC.)

Background: Livedoid vasculopathy and calciphylaxis are rare skin disorders. Large cohorts of patients have been missing so far for detailed analysis., Patients and Methods: Data from diagnosis-related groups (DRGs) of hospitalized cases of livedoid vasculopathy (n total = 1357) and calciphylaxis (n total = 699) were analysed for the years 2008-2013 concerning sex, age and frequency of diagnosis. To avoid deviations to non-relevant secondary diagnosis and due to changes in ICD-10 indices, we selected the two most recent available years 2013 and 2014 for evaluation of the accompanying diagnoses for both, livedoid vasculopathy (n = 519) and calciphylaxis (n = 324). Those were additionally evaluated as possible comorbidity., Results: The male-female ratio for livedoid vasculopathy was 2.1:1. Patients older than 45 years comprehended 74.7% of all patients with peaks between the ages of 45-50 and 70-75. Livedoid vasculopathy patients suffered from cardiovascular and renal diseases. Coding of coagulation disorders was found rarely in our analysis. For calciphylaxis, we calculated a male-female ratio of 1.7:1. Most of the patients were at an age between 65 and 80 years. Diagnosis at an age under 35 years was rare. In general, most calciphylaxis patients showed end-stage renal disease with need of dialysis and presented with the resulting complications., Conclusions: Our data analysis shows relevant comorbidity and cofactors of these rare diseases like livedoid vasculopathy and calciphylaxis in Germany by a large number of cases., (© 2017 European Academy of Dermatology and Venereology.)

Calciphylaxis is a disease in which metastatic calcification affects small- and medium-sized vessels resulting in significant dermatologic manifestations. Lesions typically occur over areas of high fat content and progress to black leathery eschars. Calciphylaxis is associated with intense pain and markedly increased risk of infection, often leading to sepsis requiring hospitalization. Diagnosis is made by clinical history and skin biopsy. Management of calciphylaxis is interdisciplinary, emphasizing factors such as primary prevention, proper wound care, pain control, and hormone and mineral balance. Although calciphylaxis carries a high mortality rate, symptomatic treatment has shown promise as a method for controlling disease progression., (© 2011 Wiley Periodicals, Inc.)

Having worked in nephrology for more than 20 years, and never encountering this condition before, we came across three patients who had been diagnosed with calciphylaxis in the space of 12 months. This condition is also known as calcific uraemic arteriolopathy and is a syndrome of medical calcification of the small arteries, which leads to painful ischaemia of the surrounding subcubitis and skin (1). Prevalence of 1-4% has been estimated in various haemodialysis populations. Also 1% per year in dialysis patients has also been suggested (3). The mortality rate for distal lesions is 23%, whilst the mortality for proximal lesions is 63% (4). The pathogenesis of this condition remains uncertain. However, vascular calcium deposition is thought to be important, and raised serum phosphate levels were associated with a substantially increased risk of calciphylaxis. Although PTH levels were high, plasma PTH was not consistently higher in patients with calciphylaxis than controls (5). The important aspect of the condition for the patients is the sheer pain and distress suffered, as well as the high mortality rate. Therefore, the implications for nurses are early detection, to allow speedy treatment to take place and involvement of the multidisciplinary team to enhance care and provide as much support as possible, thus facilitating optimal outcome and comfort.

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.

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.

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.

A 68-year-old woman presented with a painful necrotic ulcer on her right calf and necrotic breakdown of a left below-knee amputation stump as a result of calciphylaxis. No cause could be identified and corrected. Treatment comprised wound care, substituting low molecular weight heparin for warfarin, hyperbaric oxygenation and etidronate disodium, none of which were helpful and major limb amputations were considered the best option for this patient.

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 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.

Objective: Calciphylaxis, a rare disorder typically affecting renal failure patients, results in vascular calcification with subsequent skin necrosis, gangrene, and often death from sepsis. Parathyroid hormone is thought to act as a tissue sensitizer leading to these soft tissue changes. As such, parathyroidectomy is often advocated to control this complicated condition. A discussion of calciphylaxis does not exist in the otolaryngology literature, and head and neck surgeons performing parathyroidectomy should be aware of this phenomenon. This study evaluates the success of parathyroidectomy in reversing the ill effects of calciphylaxis in both our patient population and the literature., Study Design: Retrospective study and review of the literature., Methods: Five patients with calciphylaxis treated at our institution were evaluated for mortality, surgical and perioperative complications, wound healing, and predictors of patient outcomes., Results: Two patients died from sepsis and infectious complications of their calciphylaxis shortly after surgery. Of the three survivors, two later died (15 and 18 mo after surgery) from causes not directly related to calciphylaxis. The other long-term survivor required partial amputation of a leg for osteomyelitis. There was one operative complication-- wound infection requiring antibiotic therapy, drainage, and packing. Postoperative hypocalcemia required treatment in two patients. Immediate perioperative survival was more likely in patients with leukocyte counts less than 20,000 cells/mL., Conclusions: Calciphylaxis is a serious disease and patients often succumb to sepsis and infectious complications. Patients with extremely high leukocyte counts from coexistent infections may have a worse prognosis. Although a conclusive effective therapy does not exist, parathyroidectomy can be safely performed and may benefit some patients with what is often an otherwise fatal disease. The literature to date generally confirms our findings. Key Words: Calciphylaxis, parathyroid hormone, parathyroidectomy, skin necrosis, chronic renal failure.