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Competing Interests: Conflicts of interest None disclosed.

Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% ( n  = 53), followed by the thigh and gluteal regions in 7% ( n  = 4). Significant differences were identified in comorbidities, such as atrial fibrillation ( p < 0.001) and hyperparathyroidism ( p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists ( p < 0.001) , phosphate binders ( p < 0.001 ), and loop diuretics ( p < 0.01 ) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia ( p < 0.001 ), increased parathyroid hormone ( p < 0.01 ) and triglyceride levels ( p < 0.01) , hypoalbuminemia ( p < 0.01) and decreased hemoglobin values ( p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% ( n  = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.

Competing Interests: Conflicts of interest None disclosed.

Calciphylaxis, also known as Calcific uremic arteriolopathy (CUA), is a serious disorder that presents with skin necrosis due to calcification of dermal and subcutaneous adipose tissue capillaries and arterioles. The condition occurs primarily in patients with end-stage renal disease (ESRD) on dialysis, and it carries high morbidity and mortality, primarily due to sepsis, with an estimated six-month survival of approximately 50%. Although there are no high-quality studies to guide the optimal treatment approach for patients with calciphylaxis, many retrospective studies and case series support treatment with sodium thiosulfate (STS). Despite the frequent use of STS as an off-label treatment, data regarding its safety and efficacy are limited. STS has generally been considered a safe drug with mild side effects. However, severe metabolic acidosis associated with STS is a rare and life-threatening complication of STS treatment and is often unpredictable. Herein, we report a 64-year-old female with ESRD on peritoneal dialysis (PD) who presented with a profound high anion gap metabolic acidosis and severe hyperkalemia while on STS treatment for CUA. No other etiology for her severe metabolic acidosis other than STS was identified. ESRD patients receiving STS should be monitored closely for this side effect. Dose reduction, increasing the duration of infusion, or even discontinuing STS treatment should be considered if severe metabolic acidosis develops., (© 2023. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

Calciphylaxis is a rare condition characterised by painful necroses due to microvascular calcifications. It primarily affects individuals with end-stage renal disease and affected calcium-phosphate metabolism. This is a case report of a 55-year-old woman with end-stage renal disease who developed a necrotic ulcer at the breast due to calciphylaxis. Although treated with sodium thiosulfate and hyperbaric oxygen, the ulcer progressed and multiple necrotic calciphylaxis ulcers appeared. The treatment options and wound management are discussed while focusing on indications for surgical debridement., (Published under Open Access CC-BY-NC-BD 4.0. https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Calciphylaxis is a rare and devastating condition found almost exclusively in patients with end-stage renal disease. Nonuremic calciphylaxis, an even more rare diagnosis, occurs in patients with preserved kidney function. We present a fatal case of nonuremic calciphylaxis with delayed and unexpected diagnosis despite early biopsy and testing. The patient presented with a 2-month history of painful ulceration to the left leg. Early biopsy was negative for calciphylaxis. Laboratory tests were negative for renal disease and autoimmune disorders. There was elevated parathyroid hormone (96 pg/mL) 3 months after initial presentation and documented cobalamin deficiency. The patient went on to develop wounds to both legs and her thighs. A second biopsy of a left thigh wound by means of the dermatology service revealed calciphylaxis. The purpose of this case report is to raise awareness of calciphylaxis as a differential diagnosis for chronic necrotic skin ulcers, especially in patients with preserved renal function and those on warfarin therapy.

Introduction: Calciphylaxis is a serious vascular disorder characterized by calcification of tunica media, intimal hyperplasia, thrombosis, and skin necrosis. It was described in patients with renal failure (UC), although it can occur in its absence (NUC). Its risk factors are under study and its diagnosis can be complex. Over a decade ago, its mortality was estimated at 60-80%. Recent studies indicate that it has decreased (40%)., Methods: A retrospective study was carried out in the period between January 1, 2011 and December 31, 2019. The past medical record, clinical characteristics, laboratory and histopathological findings, and evolution of all patients with calciphylaxis evaluated at the Hospital Italiano de Buenos Aires were reviewed., Results: Thirty-nine patients were included. Sixtyone percent were men and 39% were NUC cases. Eightytwo percent had arterial hypertension, 66% obesity and 46% diabetes. Of those, 49% received coumarin anticoagulants. All patients with NUC and 75% with UC presented ulcers with necrosis, located more frequently on the legs. In 72% of the cases the histological diagnosis was made with one biopsy. In all the treatment was multimodal and mortality at one year was 42%., Conclusion: We observed a high proportion of patients with NUC, in relation to what is reported in the literature, and that half received vitamin K antagonists. The histological diagnosis was made with one biopsy in most of the cases, as the surgical technique for taking the sample, the Von Kossa staining and the evaluation by an expert pathologist were the key of it.

Purpose: To perform a systematic review of case reports and case series to investigate risk factors, treatment modalities, and the outcome of penile calciphylaxis., Method: We performed a systematic search of the MEDLINE and Scopus databases to identify case reports or case series of penile calciphylaxis. The patient characteristics, laboratory investigations, diagnostic modalities, treatment modalities, and outcomes were extracted. We compared clinical characteristics and treatment between patients who survived or demised and between patients with clinical improvement and those without to identify the poor prognostic risk factors., Results: Ninety-four articles were included from 86 case reports and 8 case series with 121 patients. Most of the patients were on hemodialysis (78.9%). The median time since starting dialysis was 48 months (24-96 months). Sodium thiosulfate was used to treat penile calciphylaxis in 23.6%. For surgical management, partial or total penectomy was performed in 45.5% of the patients. There was no association between sodium thiosulfate use, partial or total penectomy, and improvement in clinical outcomes. The mortality rate in patients with penile calciphylaxis was 47.8% and the median time to death was 3 months (0.75-9 months). The presence of extragenital involvement was significantly related to mortality (p = 0.03)., Conclusion: A calcified penile artery results in penile calciphylaxis, a rare vascular phenomenon associated with high morbidity and mortality. Management of penile calciphylaxis includes the medical management of risk factors, surgical debridement, or penectomy. Therefore, early prevention and diagnosis as well as immediate appropriate treatment are needed., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

This study focuses on the application of nurse-led multidisciplinary collaborative therapy (MDT) management model for calciphylaxis prevention of patients with terminal renal disease. Through the establishment of a multidisciplinary management team spanning nephrology department, blood purification center, dermatology department, burn and plastic surgery department, infection department, stem cell platform, nutrition department, pain department, cardiology department, hydrotherapy group, dermatology group, and outpatient treatment room, the distribution of duties among team members were clarified to bring out the best advantages of a multidisciplinary teamwork during treatment and nursing. For patients with calciphylaxis symptoms in terminal renal disease, a case-by-case management model was carried out with the focus on personalised problem. We emphasised on personalised wound care, precise medication care, active pain management, psychological intervention and palliative care, the amelioration of calcium and phosphorus metabolism disorder, nutritional supplementation, and the therapeutic intervention based on human amniotic mesenchymal stem cell regeneration. The MDT model effectively compensates for traditional nursing mode and could serve as a novel clinical management modality for calciphylaxis prevention in patients with terminal renal disease., (© 2023 The Authors. International Wound Journal published by Medicalhelplines.com Inc and John Wiley & Sons Ltd.)

Rationale: Calciphylaxis, known as calcific uremic arteriolopathy, is a rare cause of dry gangrene. Despite an increase in the clinical recognition of demographic characteristics and risk factors associated with calciphylaxis, it remains a poorly understood disease with high mortality., Patient Concerns and Diagnoses: We present a 45-year-old man, who was diagnosed with calciphylaxis disease, with a history of diabetes mellitus, end-stage renal disease and cirrhosis with a half-month evolution of painful dry gangrene on his glans penis and scrotum. The patient also presented with gangrene of fingers., Interventions and Outcomes: The patient and his family opted for palliative care. However, he died eventually., Lessons: This case contributed to the current understanding of calciphylaxis. Since no standard treatment is available and the prognosis remained poor, early, and accurate diagnosis of calciphylaxis is important. We here report the current case and provide data for the diagnosis and treatment of this kind of disease., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Calcific arteriolopathy involves the deposition of insoluble calcium salts in the vasculature and soft tissues, leading to ischemia, thrombosis, and cutaneous necrosis. Calcific arteriolopathy is commonly associated with ESRD but can also occur outside of ESRD, known as NUC., Objective: This article reviews the clinical management and outcomes of 3 cases of non-uremic calcific arteriolopathy., Materials and Methods: This case series describes the clinical presentation and successful treatment of NUC in a community hospital-based outpatient wound center in southeastern Wisconsin. Factors presumed to be involved included chronic kidney disease-associated mineral bone disorder, dysregulation and deficiencies of the inhibitors of vascular calcifications, autoimmune dysfunction, and chronic inflammation. All 3 patients received multimodal treatment with wound hygiene, pain management, and infection control. In addition to these interventions, case 2 received NLFU., Results: All 3 patients displayed increased tolerance of compression and debridement and successfully epithelialized with no discernible effect on long-term function or quality of life. Wound infection occurred in each case. The mean overall healing time was 20 weeks., Conclusion: The study findings suggest that successful management of NUC can be achieved using IV STS, early and aggressive infection treatment, pain control, and wound hygiene. In patients with NUC, NLFU may be an appropriate adjunct to expedite healing.

Calciphylaxis is a rare, yet underdiagnosed condition causing high mortality in patients with severe renal and cardiovascular disease. Since knowledge of the pathophysiology of calciphylaxis is limited, a differential analysis of histological alterations in patient subgroups with various comorbidities might expose different disease phenotypes and allow deeper insights into the pathophysiology of the condition. Histological markers of osteogenesis and calcification were investigated in a group of 18 patients with clinically and histologically verified calciphylaxis, using immunohistochemical staining. Analysis of staining intensity and distribution of marker proteins in histological structures was performed to evaluate distinct patterns between subgroups with different clinical comorbidities in comparison with a control group. In all cases, immunohistochemical staining for bone matrix proteins, bone-morphogenic proteins and matrix-Gla proteins co-localized with subcutaneous vascular and interstitial calcifications. Significant expression of bone-morphogenic protein-7 and active matrix-Gla protein was observed. Mortality was associated with renal comorbidities and increased expression of bone-morphogenic protein-7. However, no distinct histological patterns were found between subgroups with renal disease, warfarin intake or coexisting micro- and macro-angiopathies. The upregulation of osteogenic markers (including bone-morphogenic protein-7) plays a major role in the development of calciphylaxis. Clinical outcome correlates with kidney function and phosphate handling, suggesting different pathophysiological mechanisms. However, biopsy  at late-stage disease shows a common histological phenotype, involving enchondral ossification.

Competing Interests: The authors declare no conflicts of interest.

Aim: Calciphylaxis is a rare disease, predominantly in chronic kidney disease (CKD), characterized by high morbidity and mortality. Data from the Chinese population have been an invaluable resource for a better understanding of natural history, optimal treatments and outcomes of calciphylaxis., Methods: A retrospective study was conducted in 51 Chinese patients diagnosed with calciphylaxis at Zhong Da Hospital affiliated to Southeast University from December 2015 to September 2020., Results: Between 2015 and 2020, 51 cases of calciphylaxis were registered in The China Calciphylaxis Registry (http://www.calciphylaxis.com.cn), which was developed by Zhong Da Hospital. The mean age of the cohort was 52.02 ± 14.09 years, and 37.3% were female. Forty-three patients (84.3%) were on haemodialysis, with a median dialysis vintage of 88 months. Eighteen patients (35.3%) had a resolution of calciphylaxis and 20 patients (39.2%) died. Patients in later stages had higher overall mortality than those in earlier stages. Delay from skin lesions onset to diagnosis and calciphylaxis-related infections were risk factors in both early and overall mortality. Additionally, dialysis vintage and infections were significant risk factors in calciphylaxis-specific mortality. Among therapeutic strategies, only the use of sodium thiosulfate (STS) ≥3 courses (14 injections) was significantly associated with decreased hazard of death in both early and overall mortality., Conclusion: For Chinese patients with calciphylaxis, delay from skin lesions onset to diagnosis and infections secondary to wounds are risk factors for the prognosis of patients with calciphylaxis. Additionally, patients in earlier stages have better survival and early continuous use of STS is highly suggested., (© 2023 Asian Pacific Society of Nephrology.)

A man in his 30s, with a medical history of end-stage renal disease on haemodialysis three times a week after kidney transplant rejection, anaemia of inflammatory disease, hypertension, atrial fibrillation, hyperlipidaemia, subtotal parathyroidectomy and aortic valve replacement on Coumadin treatment, presented to our institution with glans penis pain. Examination of the penis revealed a painful black eschar with ulceration on the glans penis with surrounding erythema. CT scan of the abdomen and pelvis and penile Doppler ultrasound revealed calcifications of the abdominal, pelvic and penile blood vessels. He was diagnosed with penile calciphylaxis, a very rare manifestation of calciphylaxis characterised by penile blood vessel calcification leading to occlusion, ischaemia and necrosis. Treatment with low calcium dialysate and sodium thiosulfate was initiated with haemodialysis. Five days after the treatment started, the patient's symptoms improved., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Abstract: Calciphylaxis is an uncommon condition most often seen in patients with end-stage renal disease. It is easily mistaken for other more common conditions and requires a high level of suspicion to make a timely diagnosis. Although various treatments such as IV sodium thiosulfate and bisphosphonates have been used for management, calciphylaxis remains a condition with a high mortality that requires an interdisciplinary approach for optimal management., (Copyright © 2023 American Academy of Physician Associates.)

Calcific uraemic arteriolopathy (CUA) is a rare and poorly understood entity that typically presents with painful lesions affecting adipose-rich areas of the lower extremities. We report an unusual case of CUA presenting as presumed cellulitis of the dorsal surface of the distal lower extremity with acute development of a bullous lesion. As CUA incidence is predicted to increase due to its relationship with end-stage renal disease, recognising the full clinical spectrum of this disease is essential to promote further understanding of this disorder and exploration of additional therapeutics to limit disease morbidity and mortality., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Calciphylaxis, also known as calcific uremic arteriopathy, is a rare calcification syndrome that presents as ischemic skin necrosis and severe pain. It has a high mortality rate and is characterised by calcification of the small and medium arteries and micro-thrombosis. Calciphylaxis mainly occurs in patients with end-stage renal disease. In recent years, there have been an increasing number of cases of calciphylaxis associated with connective tissue diseases. Given the absence of clear diagnostic criteria for calciphylaxis thus far, an early diagnosis is crucial for designing an effective multidisciplinary treatment plan. In this article, we review the research progress on calciphylaxis and describe its characteristics in the context of connective tissue diseases., (© 2022 The Authors. International Wound Journal published by Medicalhelplines.com Inc (3M) and John Wiley & Sons Ltd.)

Background: Normal-renal calciphylaxis (NRC) is a devastating calcific vasculopathy characterised by refractory wounds. It is more likely to be misdiagnosed because of the absence of renal insufficiency. Failure to effectively control may lead to rapid deterioration and ultimately death. However, current knowledge of it is still inadequate., Objectives: To provide a relatively comprehensive review of NRC., Methods: Nine electronic medical databases including PubMed, Web of Science, Embase, the Cochrane Library and so on were searched from inception to April 1, 2022. Articles in which calciphylaxis was diagnosed in patients with normal renal function were included., Results: Totally 140 articles were retrieved with 187 patients (median age, 62 years [IQR, 49.63 ~ 75.70 years]; 83.42% female; 84.5% Caucasian). Vitamin K antagonism (43.32%) and diabetes (39.57%) accounted for most of the concomitant factors. Cutaneous presentations were the most common. Pathological confirmation was made in 97.86% of patients with an accuracy of 99.45%. Multidisciplinary therapeutic strategies were usually implemented while the final prognosis was not ideal, with a 6-month mortality rate of 21.10% and a 1-year mortality rate of 27.52%., Conclusion: NRC is an under-recognised disease caused by a variety of factors; meanwhile, multidisciplinary efforts are required to inform diagnostic and therapeutic decisions., (© 2022 The Authors. International Wound Journal published by Medicalhelplines.com Inc (3M) and John Wiley & Sons Ltd.)

Introduction: Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare and severe disorder that presents with skin ischemia and necrosis. Diagnosis is challenging, and even if the condition is diagnosed in the early stages, the mortality rate is exceptionally high, ranging from 45% to 80%., Case Report: A 55-year-old male with chronic kidney disease secondary to diabetic nephropathy presented with painful, severe, necrotic ulcers in the lower legs and underwent treatment with sodium thiosulfate, debridement of necrotic tissue, and topical oxygen therapy. Complete healing of the ulcers was achieved within 3 months., Conclusion: This case report raises awareness of this rare condition and details successful treatment in 1 patient.

Calciphylaxis is a life-threatening complication most often associated with chronic kidney disease that occurs as a result of the deposition of calcium in dermal and adipose microvasculature. However, this condition may also be seen in patients with acute kidney injury. The high morbidity and mortality rates associated with calciphylaxis highlight the importance to correctly diagnose and treat this condition. However, calciphylaxis remains a diagnosis that may be clinically challenging to make. Here, we review the literature on uremic calciphylaxis with a focus on its pathophysiology, clinical presentation, advances in diagnostic tools, and treatment strategies. We also discuss the unique histopathological features of calciphylaxis and contrast it with those of other forms of general vessel calcification. This review emphasizes the need for multidisciplinary collaboration including nephrology, dermatology, and palliative care to ultimately provide the best possible care to patients with calciphylaxis., (Copyright © 2022. Published by Elsevier Inc.)

Calciphylaxis is a rare and life-threatening condition in patients with end-stage kidney disease (ESKD). In this case report, we reported a 72-year-old female who had undergone aortic and mitral mechanical valve replacement 22 years ago due to rheumatic aortic and mitral stenosis. Following the valve replacement, she initiated warfarin treatment. Five years ago, she received a diagnosis of uremia and has since been undergoing regular hemodialysis. Ten months prior to her current admission, she experienced excruciating pain and was diagnosed with calciphylaxis. Additionally, an electrocardiogram revealed atrial fibrillation, while echocardiography indicated that the aortic and mitral mechanical valves were appropriately positioned, with normal perivalvular surroundings and good valve leaflet activity. No noticeable thrombosis was observed in the left atrium or left atrial appendage. Color Doppler imaging showed moderate stenosis in the lower extremity arteries, with no venous thromboembolism present. Extensive eggshell-like calcification within the arterial media was detected. The patient was managed with regular hemodialysis, symptomatic treatments (including anticoagulation and analgesia), and sodium thiosulfate. Unfortunately, symptomatic management provided limited relief, and during the one-month follow-up period, the patient passed away due to septic shock. Currently, there is insufficient conclusive evidence regarding alternative influential anticoagulants or appropriate prosthetic valve selection. For individuals with ESKD receiving maintenance hemodialysis, early identification, diagnosis, and treatment of calciphylaxis are of paramount importance.

Calciphylaxis is a rare and severe disease characterized by calcification, fibrosis, and thrombosis of small blood vessels. Although it primarily affects patients with end-stage renal disease (ESRD) on dialysis, limited cases have been reported of calciphylaxis in patients with acute kidney injury (AKI) and lupus. This case report describes the occurrence of calciphylaxis in a 35-year-old female recently diagnosed with lupus nephritis class IV and AKI requiring dialysis., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Calciphylaxis, a rapidly progressive and potentially life-threatening vascular calcification syndrome that clinically presents with persistently painful, ulcerative, or necrotizing skin lesions in multiple parts of the body, is predominantly observed in patients treated with dialysis. Early diagnosis of calciphylaxis is a key measure for reducing high disability and mortality. At present, there is no unified diagnostic standard for calciphylaxis, and there is a lack of effective early screening strategies. This paper summarized and discussed the diagnostic accuracy of calciphylaxis based on the latest research worldwide. We propose a modified strategy for the early diagnosis of calciphylaxis, which is suitable for dialysis patients to help clinicians better identify such disease and improve prognosis.

Competing Interests: Competing interests: None declared.

Nonuremic calciphylaxis is a rare condition presenting with peripheral ischemic ulcerations. Calciphylaxis is the deposition of calcium and phosphate into arteriolar walls caused by exceeding their solubility range in the blood. It is most commonly seen in patients with end-stage renal disease; however, nonuremic calciphylaxis occurs in patients with normal or mildly impaired renal function. Risk factors for nonuremic calciphylaxis include Coumadin therapy, obesity, and diabetes mellitus. Histopathologic examination of deep skin biopsy containing subcutaneous adipose tissue reveals medial calcification of dermal and subcutaneous arterioles. This diagnosis must be managed locally with wound care and systemically by control of blood calcium solubility. Avoidance of infection is critical to survival. Here we report a case of calciphylaxis in a patient with normal renal function and serum levels of calcium and phosphorus who presented with gangrene of the extremities. Increased awareness of this debilitating disease will lead to earlier diagnosis, proper treatment and improved patient outcomes., (Copyright © 2021 the American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.)

Calciphylaxis is a rare disease with severe pain and high-mortality due to cutaneous ischemic necrosis and infection that currently lacks proved effective therapies. The occurrence of calciphylaxis in end stage kidney disease (ESKD) patients is known as calcific uremic arteriolopathy (CUA), which is characterized histologically by dermal microvessel calcification, intimal fibroplasia and microthrombosis. Here we innovatively treated a severe CUA patient with human amnion-derived mesenchymal stem cells (hAMSCs). A 34-year-old uremic woman was presented with progressive, painful malodorous ulcers in buttocks and mummified lower limbs. Skin pathological features supported the diagnosis of calciphylaxis. The patient was refractory to conventional multidisciplinary symptomatic therapies. With the approval of our hospital ethics committee, she was treated with hAMSCs including intravenous and local intramuscular injection, and external application of hAMSC culture supernatant to the wound area. During 15-month follow-up, the patient had regeneration of skin and soft tissues, with improved blood biochemical, inflammatory, mineral and bone metabolic indices and immunoregulation effects. After 15-month hAMSC treatment, the score of pain visual analog scale (VAS) decreased from 10 to 0, Bates-Jensen wound assessment tool (BWAT) score decreased from 65 to 13, and wound-quality of life (Wound-QoL) questionnaire score decreased from 68 to 0. We propose that hAMSC treatment is promising for CUA patients. The therapy is potentially involved in the multiple beneficial effects of inhibiting vascular calcification, stimulating angiogenesis and myogenesis, modulating adverse inflammatory and immunologic responses, promoting re-epithelialization and restoring skin integrity.

Uremic calciphylaxis is a rare disease that affects patients with chronic end-stage renal disease. It is a pathology of the microvessels of the dermis and hypodermis which are calcified and whose thrombosis leads to skin necrosis. Calciphylaxis lesions can be distal and axial. They lead to pain, infection and are associated with denutrition and in high mortality rate (40-80% at 1 year). This general review describes the clinical and para-clinical presentations of calciphylaxis. It summarizes the current knowledge on its pathogenesis and the therapeutical options that can be proposed to improve the management and attempt to reduce the mortality of patients with uremic calciphylaxis., (Copyright © 2022 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)

Calciphylaxis is an uncommon but devastating disorder characterized by vascular calcification and subsequent cutaneous tissue necrosis. This results in exquisitely painful and slow healing wounds that portend exceptionally high morbidity and mortality. The diagnosis of this condition can be complicated because there are no conclusive serologic, radiographic or visual signs that this disease is manifesting. The differential of tissue necrosis is broad, and identifying calciphylaxis requires an adroit understanding of the risk factors and physical signs that should raise suspicion of this condition. Reviews on this subject are uncommon and lack directed commentary from disease experts on the best diagnostic approach for patients suffering from this disease. The goal of this article is to update practicing dermatologists on the current standard of care for calciphylaxis., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Calciphylaxis is a rare and devastating condition with important systemic ramifications. This second-part of our CME aims to educate the practicing dermatologist on the current standard of care once a diagnosis of calciphylaxis is confirmed or highly suspected. The key pathologic findings, as well as the role and limitations of biopsy, are reviewed. We aim to guide readers through the complex hospitalization and posthospitalization management of these medically vulnerable patients. Collaboration with other specialists will be discussed. Experimental and developing treatments are discussed, and the outlook of the condition is reported., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2022 American Academy of Dermatology, Inc. All rights reserved.)

Calciphylaxis also known as calcific uraemic arteriolopathy is a rare condition mostly seen in patients with end-stage kidney disease. We report a case of a simultaneous-kidney-pancreas transplant patient with functioning grafts developing biopsy-proven calciphylaxis in the setting of chronic inflammation. Despite several modalities of management, the patient developed progression of her disease leading to multiple amputations. This case illustrates chronic inflammation driven by persistent infection as a probable contributing factor to the development and progression of calciphylaxis in a simultaneous kidney-pancreas recipient. Calciphylaxis should be considered in the differential for a painful, non-healing ulcer even in the absence of common risk factors., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Calciphylaxis is a life-threatening cutaneous ulcerative/necrotic disease characterized by vascular calcification/occlusion. It occurs most commonly in end-stage kidney disease (ESKD), known as uraemic calciphylaxis (UC) but can also occur in patients with chronic kidney disease (CKD) and normal kidney function (nonuraemic calciphylaxis; NUC). There are few large series of NUC in the literature., Aim: To compare the clinicopathological features of UC and NUC., Methods: We retrospectively compared the clinicopathological features of 35 patients with NUC during the period 2010-2020 with those of 53 patients with UC (control group). Cases were classified as NUC in the absence of all of the following: ESKD, significant CKD (defined as serum creatinine > 3 mg/dL or creatinine clearance < 15 mL/min) and acute kidney injury requiring kidney replacement therapy or kidney transplantation., Results: NUC represented 40% of the total cases, and there was a higher number of women (P < 0.01) and a higher median body mass index (P = 0.06) compared with the control UC group. Elevated parathyroid hormone was present in 44% of patients with NUC. Most of the tested patients were positive for lupus anticoagulants (56%). NUC biopsies showed a higher rate of extravascular calcium deposits (73% vs. 47%, P = 0.03). Dermal reactive vascular proliferation was the most common dermal change (32%)., Conclusions: NUC is more common than previously reported and shows a higher predilection for obese postmenopausal women. Undiagnosed hyperparathyroidism shows a possible association with NUC. Lupus anticoagulants were positive in most patients. NUC biopsies are more likely than UC biopsies to display extravascular calcium deposition., (© 2021 British Association of Dermatologists.)

Background: Calciphylaxis is a debilitating dermatological condition associated with high rates of morbidity and mortality. Palliative care offers a multidisciplinary approach to addressing symptoms and goals of care in patients with serious medical diagnoses. Involvement of palliative services for calciphylaxis is infrequently reported in the literature. The purpose of this report is to assess rates of palliative and pain consultation for patients with calciphylaxis., Methods: This is a comprehensive, single-institution retrospective chart review of 121 eligible patients with a diagnosis of calciphylaxis treated at Mayo Clinic in Rochester, Minnesota, from 1999 to 2016. Inclusion criteria were an indisputable diagnosis of calciphylaxis based on clinical, histopathologic, and radiographic features. One hundred twenty-one patients met inclusion criteria., Results: Fifty-one patients (42%) received either a palliative (n = 15) or pain (n = 20) consultation, or both (n = 16). Patients with a palliative care consultation were younger compared with those without (mean ages 57 vs. 62 years, P = 0.046). In 104 patients (86%), psychiatric symptoms were not assessed., Conclusions: In this cohort of patients with calciphylaxis, the majority do not receive palliative and pain care consultations. Psychiatric complications are inconsistently addressed. These observations highlight practice gaps in the care of patients with calciphylaxis., (© 2021 the International Society of Dermatology.)

Competing Interests: The authors report no conflicts of interest.

Background: Calcification can be seen vascular, peritoneal and also subcutaneous. Subcutaneous calcifications are seen in calciphylaxis, which is a rare but life-threatening condition with painful skin lesions and varying stages of skin necrosis, mostly described in patients on dialysis., Case Description: A 65-year old man presented at the emergency room with complaints of abdominal pain. He had a long history of chronic kidney disease, currently on hemodialysis after previous peritonitis from peritoneal dialysis. Physical examination shows painful plate-like areas of indurated skin on the abdomen with possible central spots of necrosis, suspicious for calciphylaxis. CT-scan showed calcifications on multiple sides. Unfortunately it was already in an advanced stage. He died a few days after hospitalization., Conclusion: Recognition of calciphylaxis at an early stage is important to prevent and reduce mortality by elimination of risk factors, prevention of infection, pain relief, increasing frequency and length of dialysis, and administration of sodium thiosulfate.

Calciphylaxis is a rare and serious complication of chronic renal failure characterized by vascular calcium overload. It has a high mortality rate. Penile calciphylaxis is an extremely rare condition of penile necrosis due to calciphylaxis of the penile arterioles. Presented here is a severe case of systemic calciphylaxis initially presented as penile necrosis treated with sodium thiosulfate and amputation.

Background: Calciphylaxis and the arteriolosclerotic ulcer of Martorell (ASUM) represent two entities of cutaneous calcific arteriolopathies. Their differential diagnosis can be challenging, given similarities in their clinical and histological presentation. Calcification patterns have been proposed as a possible discriminative histological criterion, however, a systematic microstructural comparative analysis is lacking., Objectives: The study aimed at a systematic comparative microstructural analysis of the calcification patterns in calciphylaxis versus ASUM., Materials & Methods: Skin biopsies of patients with leg ulcers due to calciphylaxis (20) and ASUM (69) diagnosed at three European wound care centres (Vienna, Bern, Zurich) were included. The extent of calcification, arteriolar calcification pattern and presence of extra-arteriolar calcification were assessed., Results: All calciphylaxis and most ASUM patients (77%) presented with arteriolar calcification. Although the mean number of calcified vessels and the proportion of calcified area were significantly higher in calciphylaxis specimens (p = 0.003 and p = 0.0171), there was no significant difference in the pattern of arteriolar calcification (p = 0.177). Interestingly, extra-arteriolar calcification was detected in the majority of both calciphylaxis (93.3%) and ASUM samples (85.2%, p = 0.639). Notably, Alizarin Red S staining was superior to H&E for the detection of calcifications of both entities (p = 0.014 and p < 0.0001), and to von Kossa staining for ASUM samples (p = 0.0001). However, no differences could be observed between cases with uraemic and non-uraemic calciphylaxis or ulcerations located on the upper and lower leg., Conclusion: Our results indicate that extra-arteriolar calcification is not only present in calciphylaxis, but can also be detected in ASUM suggesting a lack of specificity for this finding. However, more specific calcification stains, such as Alizarin Red S, should be used in suspected cases, as calcifications may be overlooked using conventional H&E staining.