Case Of Calciphylaxis Research Articles

#2214 Incidence, risk factors and outcome of calciphylaxis in Iceland

Abstract Background and Aims Calciphylaxis is a rare and serious medical condition that primarily affects patients with end-stage kidney disease (ESKD). The disorder is characterized by calcium deposition in blood vessels and soft tissues, resulting in skin ulceration and tissue necrosis. The aim of the study was to investigate the incidence, risk factors, and outcomes of calciphylaxis in Iceland. Method This was a retrospective case-control study that included individuals diagnosed with calciphylaxis in 1990-2022. Patients were identified by searching electronic medical records (EMR) for the ICD-10 diagnosis codes E83.5 and L94.2, SNOMED histology codes T01000-T03900 in combination with M55400, M55500 or M54000-M54200, documented sodium thiosulfate (STS) therapy, and by consultation with practicing nephrologists. Data on medical history, medication use, and survival were obtained from EMR. A control group of ESKD patients was matched (1:1) based on age, sex, and duration of dialysis. Total annual incidence was calculated per 1,000,000 population of Iceland each year, and for ESKD patients per 10,000 dialysis patients at the end of each year. Poisson regression was used to estimate changes in incidence. Groups were compared using non-parametric tests and survival estimated by Kaplan-Meier method and Cox regression. Results Twenty-one cases of calciphylaxis were identified, of which 18 (86%) had ESKD. The average incidence was 2/1,000,000/year. For ESKD patients, the median (interquartile range, IQR) age was 69 (66-75) years, and 50% were women. Three patients (17%) had not yet initiated dialysis at the time of diagnosis and the median duration of dialysis was 0.12 (IQR 0.0-2.8) years. The average incidence of calciphylaxis was 75 per 10,000 dialysis patients/year, increasing by 9.0% annually (Fig. 1). No significant differences between calciphylaxis cases and controls were observed for comorbid diseases or other risk factors. The median body mass index of cases was 29.5 (IQR 27.1-33.7) compared with 27.6 (IQR 24.3-30.6) kg/m2 in controls (p=0.20), and 50% of cases and 22% of controls (p=0.16) were on warfarin treatment at diagnosis. Fifteen patients received treatment with sodium thiosulfate (STS). Three of 6 patients received STS therapy before 2016 and all 12 patients after 2016. One-year mortality of ESKD patients with calciphylaxis was 44%. The median survival was 134 days in 1990-2016, increasing to 644 days after 2016. Survival difference between cases and controls was significant (p=0.04) by log rank test, while adjusted Cox regression did not show a statistically significant association between calciphylaxis and death (HR 2.0 (95% CI, 0.67-6.1)). Conclusion The incidence of calciphylaxis in Iceland seems to be increasing and is high compared to incidence rates reported in other countries. Survival, although poor, appears to have improved in recent years. The small sample size is a limitation and further research will require collaborative efforts to generate large patient samples.

S3544 A Rare Case of Calciphylaxis in a Patient With Cirrhosis and Chronic Kidney Disease That Resolved After Simultaneous Liver-Kidney Transplantation

S3544 A Rare Case of Calciphylaxis in a Patient With Cirrhosis and Chronic Kidney Disease That Resolved After Simultaneous Liver-Kidney Transplantation

Clinical features and outcomes of calciphylaxis in Chinese patients with chronic kidney disease.

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. 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. 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. 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.

Calciphylaxis and its co-occurrence with connective tissue diseases.

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.

S2930 Calciphylaxis Due to Alcoholic Liver Disease

Introduction: Advanced liver disease often has multiorgan involvement, including dermatological findings. These range from spider angiomas to palmar erythema. Here we present a case of calciphylaxis as a manifestation of decompensated alcoholic liver disease. Case Description/Methods: A 38 year-old female with decompensated alcoholic cirrhosis (portal vein thrombosis, ascites, hepatic encephalopathy, and hepatic hydrothorax) reported to the emergency room complaining of new right thigh wounds. Her vital signs were unremarkable and her exam demonstrated ascites and bilateral lower extremity edema with right medial thigh erythema, induration, and superficial ulceration (Figure 1). Labs showed sodium 125 mEq/L, Creatinine 0.95 mg/dL, WBC 16.2 x109, and total bilirubin 1.6 mg/dL. CT of her thigh demonstrated bilateral, symmetric subcutaneous induration consistent with anasarca and possible superimposed cellulitis. She was admitted and treated with IV antibiotics. As the lesions became duskier and more painful dermatology was consulted who initially were concerned for a vasculopathic process versus calciphylaxis. The patient was placed on empiric treatment for calciphylaxis with sodium thiosulfate. Skin biopsies were performed and showed non-uremic calciphylaxis (Figure 2). The patient then developed an AKI thought to be from hepato-renal syndrome and was trialed on albumin, midodrine, and octreotide with no improvement in renal function. She then developed acidosis, eventually requiring a bicarb drip, and her wounds continued to progress (Figure 1). When she started becoming repeatedly hypotensive her family transitioned her to comfort care and she was discharged to hospice. Discussion: Calciphylaxis is a rare disease that was first described in 1961 by Selye et al and is largely thought to be related to uremia. Here we describe a case of calciphylaxis in a patient with alcoholic liver disease and initial normal renal function. Our review of the literature found fewer than 11 case reports of calciphylaxis associated with liver disease. The mechanism in these cases appears to be poorly understood though is believed to be connected to protein C and protein S deficiency. As in those with advanced renal disease, calciphylaxis has a poor prognosis with high mortality often reported as greater than 50% even despite treatment. Thus it is important that clinicians recognize its association with alcoholic liver disease to aid in diagnosis and ensure prompt initiation of treatment.Figure 1.: A: Right medial thigh wound B: High power of calcium deposits and thrombus (arrow) within the subcutaneous fat C: corresponding von kossa stain (arrowhead).

Characterization of Risk Factors for Calciphylaxis in Hemodialysis Patients in the Fraser Health Renal Program – A Matched Case-Control Retrospective Review

Introduction: Calciphylaxis is a lethal and rare disease characterized by ischemic and necrotic skin lesions caused by vascular calcification of adipose tissue. There have been many risk factors analyzed in the literature; however, the pathogenesis of calciphylaxis is still not well understood and treatment options are limited due to the lack of interventional studies. Our objectives were to describe risk factors, prevalence, incidence, and outcomes for calciphylaxis in hemodialysis patients within the Fraser Health Renal Program. Methods: This was a retrospective matched case-control study of hemodialysis patients within the Fraser Health Renal Program. Hemodialysis patients with calciphylaxis were matched to hemodialysis patients without calciphylaxis in a 1:2 ratio for age and sex from September 2, 2017 to July 3, 2020. Findings: There was a total of 40 calciphylaxis cases matched to 80 controls. In the univariate analysis, peritoneal dialysis, higher body mass index, lower serum iron, lower transferrin saturation, sevelamer, cinacalcet, warfarin, iron (PO), and insulin were associated with increased risk of calciphylaxis. In the multivariate analysis, only peritoneal dialysis, serum iron, sevelamer, and warfarin were identified as significant and strong risk factors associated with calciphylaxis. A low prevalence of 1.9% and high mortality rate of 57.5% at 12 months was found for calciphylaxis cases. Discussion: Significant risk factors associated with calciphylaxis were peritoneal dialysis, serum iron, sevelamer, and warfarin. Future studies should further investigate the impact of minimizing exposure to these risk factors to reduce calciphylaxis development.

217 Calciphylaxis arising following bariatric surgery

Calciphylaxis is a rare, highly morbid disease leading to vascular calcification and cutaneous necrosis, typically associated with end-stage renal disease (ESRD). While the pathogenesis and risk factors of calciphylaxis are poorly understood, some cases of calciphylaxis in patients who had previously undergone gastric bypass have been reported. The objective of this study is to report cases and describe an association between nutritional deficiencies in the context of bariatric surgery and calciphylaxis. Retrospective study in one large academic hospital that serves as a referral center for calciphylaxis in Boston, Massachusetts between 2012-2018 involving 11 patients who had bariatric surgery prior to calciphylaxis. Median time between bariatric surgery and calciphylaxis diagnosis was 2 years and 2 months, (range 7 months – 30 years). Parathyroid hormone levels were significantly higher in patients with ESRD compared to those with non-uremic calciphylaxis (p=0.036). Corrected calcium levels were significantly higher in patients who had malabsorptive compared to restrictive procedures (p=0.01). Limitations include retrospective nature, sample size, and missing weight loss data from remote bariatric surgical cases. Recognition of risk factors for calciphylaxis and careful evaluation of therapies for other comorbidities in patients who have undergone bariatric procedures is essential to improve outcomes for this rare but problematic disease.

MO751: Calciphylaxis is Characterized by Pyrophosphate Deficiency in Dialysis Patients: A Pilot Study

Abstract BACKGROUND AND AIMS Calciphylaxis is a rare but devastating disorder predominantly affecting patients with end-stage kidney disease [1]. Patients with calciphylaxis present with painful ischemic skin lesions caused by microvascular occlusion secondary to calcification, intimal hyperplasia, and thrombosis. In experimental models and in genetic disorders of mineralization, deficiency of pyrophosphate (PPi) results in vascular calcification and neointimal formation [2–4]. We investigated the role of PPi deficiency in the development of calciphylaxis and explored whether PPi levels predict outcomes among patients with calciphylaxis. METHOD As part of the ongoing national-level calciphylaxis registry work (ClinicalTrials.gov Identifier: NCT03032835) [5], we prospectively enrolled 14 dialysis-dependent patients with calciphylaxis (cases) and 7 dialysis-dependent patients without calciphylaxis (controls) matched for age, sex and race. Clinical data and blood samples were collected at enrolment. PPi levels were measured using an ATP Sulfurylase/Luminescence-Based Method for the quantification of inorganic PPi in human plasma and were compared between cases and controls using the Kruskal–Wallis test. Associations between PPi levels and clinical outcomes (ulcer formation, number of skin lesions and 1-year mortality) were explored. RESULTS The average age of calciphylaxis cases and controls was 62 and 61 years, respectively; 53% were women and 53% were Caucasian. The prevalence of diabetes mellitus, obesity, coronary artery disease and peripheral arterial disease were 71%, 23%, 29% and 53%, respectively and comparable between cases and controls. Warfarin exposure was present in 19% of cases. Most patients with calciphylaxis had ulcerated skin lesions involving abdominal wall and/or thighs. Median number of calciphylaxis skin lesions was 2 [interquartile range (IQR) 1–4]. Serum levels of calcium, phosphate, intact parathyroid hormone, 25-OH vitamin D and alkaline phosphatase were similar between cases and controls. Compared with controls, however, patients with calciphylaxis had lower plasma PPi levels [0.32 μM (IQR 0.19–0.70) versus 0.64 μM (IQR 0.46–1.08); P = 0.03]. Plasma PPi levels demonstrated a modest correlation with skin lesion count (r = –0.35) but were similar between patients with and without ulcer formation. Lower plasma PPi levels predicted 1-year mortality (Fig. 1). CONCLUSION In this pilot study, dialysis-dependent patients with calciphylaxis had lower levels of plasma PPi compared with controls. Plasma PPi levels predicted 1-year mortality among patients with calciphylaxis. Additional data from a larger study and longitudinal assessments of PPi levels are needed to support future clinical intervention trials targeting the PPi pathway in calciphylaxis.

Identifying subcutaneous tissue microcalcification by Fluo-3 AM imaging in cutaneous calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is typically characterized by subcutaneous tissue calcification and excruciatingly painful cutaneous lesions with high mortality. It is critical for dermatologists to make early diagnosis and appropriate management, yet currently only 56% of calciphylaxis cases are correctly diagnosed by conventional histological stain1. Specially, the identification of subtle calcium deposits of subcutaneous can be challenging but is believed crucial for early diagnosis of calciphylaxis2. More sensitive calcification staining is in high demand. In this study, Fluo-3 AM was found to be a rapid, sensitive and reliable fluorescent probe for the detection of calcium deposits and could be a promising diagnostic tool for calciphylaxis.

Case series of nonhealing wound and calciphylaxis in dialysis patients from Qatar (Treated with Multi-Interventional Strategy)

Calciphylaxis is a rare and serious disorder that presents with skin ischemia and necrosis and is characterized histologically by calcification of dermal arterioles. It is also called “calcific uremic arteriolopathy” (CUA). It has a poor prognosis and often occurs in patients undergoing dialysis. We present six cases of calciphylaxis associated with a wide range of symptoms, including nonhealing thigh, abdominal wall, breast and penile ulcer. We report our experience of a multi-interventional strategy in treating patients with calciphylaxis and nonhealing ulcers.

Teriparatide-associated calciphylaxis: a case series.

Calciphylaxis is a rare and potentially fatal small-vessel occlusive disease in which the tunica media becomes calcified, endothelial cells proliferate, and the tunica intima becomes thickened and fibrotic. Calciphylaxis typically occurs in the setting of end-stage renal disease with secondary hyperparathyroidism and elevated calcium-phosphorus product. The estimated incidence of calciphylaxis in dialysis or kidney transplant patients is 1 to 4%; however, the incidence of non-uremic calciphylaxis is unknown. We assessed postmarketing adverse event reports to further characterize cases of calciphylaxis associated with teriparatide. We searched for cases of teriparatide-associated calciphylaxis in the literature (EMBASE, PubMed) and those reported to FDA, including the FDA Adverse Event Reporting System, through March 31, 2021. We included calciphylaxis cases following teriparatide exposure of < 2years. Twelve cases described teriparatide-associated calciphylaxis. The median age was 81 (range 47-86) years. Eleven cases reported confirmatory biopsy and/or imaging. The median time-to-onset of calciphylaxis following teriparatide initiation was 3.5 (range 1-20) months. Three cases reported hospitalization, of which one resulted in death due to progression of the lesions. All cases had multiple risk factors (mean (SD), 4.5 (1.0)) including concomitant medications associated with calciphylaxis (12), female sex (11), and/or underlying autoimmune disease or other inflammatory disorder (10). We believe that exposure to teriparatide, coupled with underlying risk factors, may have triggered new-onset calciphylaxis. Expedited diagnosis and management by a clinician are important because calciphylaxis may be life-threatening and early intervention may improve outcomes.

The effect of phosphate binder therapy with sucroferric oxyhydroxide on calcification propensity in chronic haemodialysis patients: a randomized, controlled, crossover trial.

Calcification propensity is associated with the risk for cardiovascular events and death in end-stage renal disease patients. Here we investigated the effect of lowering serum phosphate with oral phosphate binder therapy on calcification propensity. We performed an open-label, randomized, controlled, crossover study in chronic haemodialysis patients with hyperphosphataemia. Patients (n = 39) were randomized in a 1:1 ratio to either low-dose (250 mg/day) sucroferric oxyhydroxide (SO) followed by high-dose (2000 mg/day) SO or vice versa, with washout phases before and after SO treatment. The primary endpoint was changed in calcification propensity as measured by calciprotein particle formation time (T50 test) between washout and high-dose SO treatment in patients with ≥85% adherence to the prescribed SO dose (per-protocol analysis). In the primary per-protocol analysis (n = 28), 2000 mg/day SO treatment resulted in a mean increase in T50 of 66 min (95% CI 49-84 min, P < 0.0001), from 243 ± 63 to 309 ± 74 min compared with phosphate binder washout. Serum phosphate decreased from 2.28 ± 0.5 to 1.63 ± 0.43 mmol/L (P < 0.0001). SO at 250 mg/day did not influence T50 (P = 0.4) or serum phosphate concentrations (P = 0.9) compared with phosphate binder washout. The secondary intention-to-treat analysis (n = 39) showed similar results: an increase in T50 of 52 min (95% CI 31-74 min, P < 0.0001) and a decrease in serum phosphate from 2.18 ± 0.5 to 1.64 ± 0.46 mmol/L. No major adverse cardiovascular event, case of calciphylaxis or death occurred during the study. Phosphate binder treatment with SO improves serum calcification propensity of haemodialysis patients and might lead to improved outcomes.

A case of calciphylaxis with an unfavorable outcome

A case of calciphylaxis with an unfavorable outcome

The Australian Calciphylaxis Registry: reporting clinical features and outcomes of patients with calciphylaxis.

Calciphylaxis is a rare disease, predominantly affecting patients with chronic kidney disease (CKD) and associated with significant morbidity and mortality due to progressive cutaneous calcification, necrotic ulceration and infection. Clinical registries have been established to better understand the risk factors, optimal treatments and disease outcomes of calciphylaxis. We established a prospective, Internet-based clinical registry for the online notification of calciphylaxis cases in Australia. Seven institutions participated, with data recorded on patient characteristics, biochemical parameters, treatments and disease outcomes. Between 2014 and 2019, 47 cases of calciphylaxis were registered. The mean patient age was 66 ± 11 years and body mass index was 35 ± 9 kg/m2, with a higher proportion of females (51%). Eighty-seven percent of patients had end-stage kidney disease (ESKD), with 61% on hemodialysis or hemodiafiltration, with a median dialysis vintage of 4.8 [interquartile range (IQR) 1.7-7.4)] years. Five patients had CKD not requiring dialysis and two were kidney transplant recipients. Diabetes was present in 76% of patients and the cause of ESKD in 60%; 34% received vitamin K antagonists (VKAs) before diagnosis. The median parathyroid hormone level at diagnosis was 32 (IQR 14-50) pmol/L. The most common site of calciphylaxis was the lower limbs (63%), with 19% of patients having more than one area involved. Ten patients (22%) had a resolution of calciphylaxis and 25 died, with 50% mortality at a median of 1.6 (IQR 0.2-2.5) years from diagnosis. The Australian Calciphylaxis Registry highlights risk factors for calciphylaxis, including diabetes, obesity and VKA use. Resolution of calciphylaxis is uncommon despite multimodal therapy and mortality from calciphylaxis in the first year following diagnosis remains high.

Diffuse dermal angiomatosis associated with calciphylaxis: A 5-year retrospective institutional review.

Although diffuse dermal angiomatosis (DDA), a rare acquired reactive cutaneous vascular disorder, has been previously reported in association with calciphylaxis (CP), the clinical significance of this relationship has not yet been elucidated. A total of 24 cases of CP diagnosed from 2013 to 2018 were retrospectively reviewed for the presence of associated DDA. Pertinent clinical information for each patient was also collected, and statistical analysis was performed using multivariable logistic regression, Student t test and Fisher exact test. African American race and comorbid congestive heart failure were the only variables that demonstrated independent, statistically significant association with the presence of DDA. End-stage renal failure, diabetes mellitus, immunosuppressive and hypercoagulable states, arrhythmia, body mass index, hypertension, coronary artery disease, patient age, duration of CP symptoms, gender, time interval from biopsy to death, anticoagulation therapy and sodium thiosulfate administration at the time of biopsy did not demonstrate a statistically significant association with DDA. DDA does not appear to be associated with disease severity or prognosis in cases of CP; however, in our population CP with concurrent DDA was more prevalent in African Americans and individuals with congestive heart failure.

The Perfect Storm: A Case of Non-Uremic Calciphylaxis.

Calciphylaxis is a severe condition associated with chronic kidney disease. We describe a rare case of non-uremic calciphylaxis in a 79-year-old woman with prosthetic aortic and mitral valves, chronic kidney disease and anaemia, who presented with painful skin lesions on her left leg. She was taking warfarin, calcium and iron supplements, and had been recently diagnosed with oligoarthritis and started on prednisolone, all factors associated with calciphylaxis. Despite treatment, the lesions rapidly progressed to large ulceration and sepsis, and pain management was challenging. Calciphylaxis was confirmed following a second skin biopsy but the patient died from septic shock and multi-organ failure. This case highlights the challenges associated with the diagnosis and treatment of calciphylaxis despite a high level of suspicion.LEARNING POINTSThe number of reported cases of calciphylaxis, once considered very rare, is increasing.Physicians should be aware of new information concerning the condition.Treatment includes a low antibiotic threshold, comprehensive pain management and efficient wound care.

Calciphylaxis — pathogenesis and clinical picture

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare vascular disease caused by deposition of calcium salts in vessel walls, accompanied by coagulation disorders, which leads to extensive skin ulceration and soft tissue necrosis. Typical cases of calciphylaxis are usually described in patients with renal failure, though it is currently known that approx. 1/10th of all cases stem from other causes, including systemic inflammation, metabolic disorders and endocrine disorders. This article shows an overview of the most important pathophysiological phenomena which accompany calciphylaxis, characterizes the clinical picture of the disease and presents available approaches to therapeutic management. Forum Reumatol. 2019, tom 5, nr 1: 14–20

Calciphylaxis in a renal transplant recipient with normal graft function: A case report and review of literature

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare, life-threatening complication of end-stage renal disease. However, it may also occur in patients without renal failure, the nonuremic calciphylaxis. Most patients present with painful skin ulcers. Delayed diagnosis can lead to sepsis-related morbidity and mortality. Aberrations in calcium, phosphate, and parathyroid hormone physiology are common and biopsy is diagnostic. Early diagnosis and correction of vascular and metabolic aberrations may assist in healing as may the use of sodium thiosulfate and hyperbaric oxygen therapy. We report on successful treatment of a case of calciphylaxis in a renal transplant recipient with normal allograft function.

Histopathology of Calciphylaxis: Cohort Study With Clinical Correlations.

Calciphylaxis is a rare, painful, and life-threatening condition with a high mortality rate. Although the etiology of calciphylaxis is not well understood, it has been proposed that calcium deposition within and around subcutaneous vessels restricts blood flow chronically, thereby predisposing the patient to acute pannicular and dermal thrombosis. Given increasing recognition of the role of hypercoagulability in calciphylaxis, this retrospective cohort study sought to evaluate the presence of thromboses and dermal angioplasia in calciphylaxis. Moreover, we aimed to validate previous observations about the histopathology of calciphylaxis compared with skin biopsies from patients with end-stage renal disease but without calciphylaxis. After a meticulous clinical chart review, we assessed the corresponding skin biopsies for the presence of vessel calcification, thromboses, and dermal angioplasia in skin biopsies from patients with calciphylaxis (n = 57) and compared with those from patients with end-stage renal disease but without calciphylaxis (n = 26). Histopathologic findings were correlated with clinical features such as chronic kidney disease, diabetes, or associated malignancy. Our results validated a prior observation that calciphylaxis was significantly more likely to show calcification of dermal vessels and diffuse dermal thrombi. This study reports the frequent finding of dermal angioplasia, a potential marker of chronic low-grade ischemia, as another frequent microscopic finding in calciphylaxis. Among cases of calciphylaxis, histopathologic changes in patients with chronic kidney disease were indistinguishable from those in patients without chronic kidney disease, thereby implying a final common pathogenic pathway in both uremic and nonuremic calciphylaxis. In future, larger, prospective studies may be useful in validating these findings.

Non-uraemic calciphylaxis: a diagnostic and management challenge for the burns team

Calciphylaxis is a rare systemic condition usually seen in patients with end-stage renal disease. It is characterised by pathological calcification of dermal blood vessels, causing ischaemia and necrosis in the skin and subcutaneous fat. The lesions are usually small but, in extreme cases, may be very extensive and may mimic necrotising fasciitis, prompting extensive surgical debridement. We describe the challenges faced in managing such a case. A 38-year-old female patient was admitted to another hospital with widespread progressive truncal skin necrosis. A provisional diagnosis of necrotising fasciitis was made, and she underwent debridement of 32% of her total body surface area (TBSA). However, her condition rapidly deteriorated and she was transferred to our burn centre for further management. Diagnosis of calciphylaxis was based on clinical features and confirmed by tissue biopsy. Characteristic findings of progressive skin and fat necrosis at the wound margins with healthy underlying fascia and muscle were recognised from previously treated cases of calciphylaxis; however, the absence of renal disease proved misleading. Non-uraemic calciphylaxis is very rare but can, as in this case, be associated with alcoholic liver disease. At our centre, the patient required prolonged ventilation and supportive treatment in burns critical care, combined with intensive wound management, repeated episodes of debridement, temporary wound cover using human skin allografts and reconstruction using split-thickness autograft. We report this case to alert others to this rare condition as a possible diagnosis in patients presenting with extensive panniculitis. We discuss associations, diagnostic difficulties, novel therapies and the importance of multidisciplinary care in managing these complex cases. Level of evidence: Level V, diagnostic study.