Coexistence of nephrogenic systemic fibrosis and calciphylaxis in a gadolinium-naïve, chronic haemodialysis patient.

We present a case of a man in his 40s who was on haemodialysis for over 20 years presenting with rapidly progressive decline in mobility, associated with fixed flexion deformities of joints and peau d'orange appearance of skin together with areas of ulceration that was concerning for calciphylaxis. Skin biopsies were consistent with both nephrogenic systemic fibrosis and calciphylaxis. He has never had exposure to gadolinium-based contrast agent. His treatment included daily dialysis sessions, which were challenging due to vascular access issues and three times weekly sodium thiosulfate. He rapidly declined in hospital and died within 2 weeks of presentation while being treated for a hospital-acquired pneumonia.

Atypical Warfarin-Induced Calciphylaxis Outside a Typical Presentation of End-Stage Renal Disease on Hemodialysis.

We present a case report highlighting a 47-year-old woman who developed warfarin-induced calciphylaxis. She initially developed bilateral leg wounds secondary to restraint straps from helicopter transportation to a higher level of care for treatment of critical aortic stenosis. She was started on warfarin following surgical implantation of a mechanical aortic valve. After her wounds failed to heal, a punch biopsy of the wounds demonstrated ulceration, altered vasculature, and soft tissue calcification. The pathology confirmed the clinical concern for calciphylaxis, which is most often diagnosed in patients with a history of end-stage renal disease on hemodialysis. However, our patient did not demonstrate evidence of renal disease prior to the onset of calciphylaxis. Her wounds began to heal after treatment with sodium thiosulfate and changing her anticoagulation from warfarin to rivaroxaban.

Erythema nodosum with incidental calciphylaxis secondary to zoledronic acid and denosumab.

Erythema nodosum (EN) is the most common clinical presentation of panniculitis, an inflammatory process that affects subcutaneous cellular tissue, characterized by the acute appearance of painful erythematous nodules predominantly in the lower extremities. An unusual case of EN is presented below, secondary to the administration of zoledronic acid (ZA) and denosumab, in which incidental histopathological findings of calciphylaxis were also found.

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 < 2 years. 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.

Skin necrosis and calcifications after extravasation of vancomycin: a localised form of calciphylaxis?

Vancomycin is a tricyclic glycopeptide antibiotic produced from Streptococcus orientalis. There is much variation in the literature with regard to the recommended dose, dilution rate and type of infusion. Given the vesicant properties of vancomycin at supratherapeutic doses (>10mg/ml), tissue damage including blistering and necrosis have been reported. We report a rare case of bilateral cutaneous necrosis induced by accidental extravasation of vancomycin when being intravenously administered. The skin surrounding the injection site was marked by the appearance of subcutaneous calcifications. The development of iatrogenic skin calcinosis has not yet been described for the extravasation of vancomycin. The mechanism underlying the calcinosis observed in our case remains unclear, but we hypothesised a form of localised calciphylaxis induced by a local triggering factor. The ulcers progressed to re-epithelialisation following necrosis debridement and local conservative treatments. Given the increased prevalence of meticillin-resistant Staphylococcus aureus, which has prompted clinicians to gradually increase vancomycin dosage, clinicians should be aware of the high risk of skin toxicity in cases of vancomycin high-dose extravasation.

Breast fat necrosis secondary to warfarin-induced calciphylaxis, a rare mimicker of breast cancer: A case report and a review of literature.

Breast fat necrosis (BFN) is usually a benign inflammatory response to breast trauma. However, an extremely rare cause of fat necrosis is calciphylaxis, a calcification of small- and medium-sized arteries causing thrombosis and ischemia. It is classified into (A) uremic (B) nonuremic-induced calciphylaxis. Calciphylaxis has been reported to be encountered in different parts of the body. However, to the best of our knowledge there is only one case in the English literature of BFN 2ry to warfarin-induced calciphylaxis. We report a 65-year-old female, known case of atrial fibrillation on warfarin, presented with a left breast mass of 4-month duration. The mass was painful and progressively enlarging. Examination of the left breast showed 7 × 4 cm mass, spanning from 10-2 o'clock, free from surrounding structures, with preserved overlying skin. However, the mass was not visualized on mammogram. Ultrasound showed a left breast lobulated hypoechoic mass containing a hyperechoic component. Biopsy showed fat necrosis. After 1 month, she presented with ulceration of the overlying skin. After wide local excision, histopathology demonstrated a calciphylaxis-induced fat necrosis. Considering the patient's background, the diagnosis was BFN secondary to warfarin-induced calciphylaxis. Hence, the warfarin was shifted to Rivaroxaban, 6 months follow-up showed no evidence of recurrence. In conclusion, the rarity of nonuremic calciphylaxis is reflected on the delay of diagnosis in some of the reported cases and the lack of grading system used to guide the management of such difficult wounds. However, keeping a high index of suspicion is important whenever such wounds are encountered with presence of risk factors other than end-stage kidney disease.

[Iatrogenic non uremic calciphylaxis: A case report]. / Calciphylaxie non urémique iatrogène : une nouvelle observation.

Calciphylaxis is a rare and severe condition, characterized by calcification and thrombosis of small vessels, mainly affecting the skin. It is most often described in patients with end-stage renal disease on dialysis. Rarer cases of non-uremic calciphylaxis are reported. The prognosis is grim and the treatment is not well codified. Sodium thiosulfate has been used for more than a decade in the treatment of uremic calciphylaxis and has been shown to be effective. Its use in non-uremic cases has been reported in a few rare observations. Rheopheresis is a technique very recently used as an adjuvant treatment in uremic calciphylaxis. We describe a case of non-uremic calciphylaxis in a patient with normal renal function and with calcium supplementation. Sodium thiosulfate was introduced, then discontinued due to the patient's poor tolerance for this treatment. Rheopheresis was then used and allowed the acceleration of healing process and a significant reduction in pain. These two treatments are promising, larger studies are needed to establish their effectiveness in non-uremic calciphylaxis.

Non-uremic Calciphylaxis: A Rare and Late Adverse Reaction of Warfarin.

BACKGROUND: Calciphylaxis is a complex dermatological lesion of micro vascular calcification that is typically presented as panniculitis with gangrenous painful lesions having uremic and non-uremic causes. CASE REPORT: We present a case of a 48-year old male with a history of paroxysmal atrial fibrillation and hypertension taking amlodipine 5 mg and warfarin 5 mg daily for the last 26 months. The patient had a 6- months history of painful swelling followed by necrotic skin ulcer over the right leg. His remarkable examination findings were right leg tender ulcer with surrounding erythema and secondary sepsis. His hemogram, metabolic profile and connective tissue diseases work up were unremarkable except leucocytosis and raised inflammatory markers. His local part radiological and skin biopsy findings were suggestive of calciphylaxis. RESULTS AND CONCLUSION: In our case, warfarin and amlodipine were culprit drugs for the lesion, but Naranjo score (warfarin 7and amlodipine 1) speculate warfarin as a probable adverse reaction of warfarin. The lesion was cured with local wound treatment after discontinuation of warfarin. The physician should be aware of this rare cutaneous disorder of systemic origin for proper management.

Resolution of acenocoumarol-associated calciphylaxis with drug withdrawal.

Calciphylaxis is a syndrome of cutaneous ischaemic necrosis and ulceration due to arteriolar calcification with subsequent thrombosis, which rarely presents in patients without terminal kidney disease. Recently, several reports of coumarins-associated calciphylaxis have stressed the relevance of anticoagulant therapy as an important risk factor for the development of this condition. We report five cases of acenocoumarol-associated, biopsy-proven calciphylaxis in women aged between 64 and 92 years. The drug had been prescribed for atrial fibrillation and was taken without interruption from 14 to 224 months. Lesions were present for months in all cases and were resistant to multiple therapeutic options, but they resolved only with simple wound care measures 6-14 months after changing the anticoagulant therapy.

Calciphylaxis-as a drug induced adverse event.

INTRODUCTION: Calciphylaxis is a rare but devastating disease with a mortality rate up to 50% in 1 year. It is characterized by profoundly painful ischemic skin lesions and vascular calcification that affects predominantly patients with end stage renal disease. The use of certain medications is an important modifiable risk factor in calciphylaxis and discontinuation of these is a mainstay of treatment. AREAS COVERED: This review will provide an overview of calciphylaxis and will focus on how certain therapeutic agents can affect the risk of calcification and associated thrombosis, key processes involved in the development of calciphylaxis. EXPERT OPINION: Calciphylaxis treatment requires a multi-modal approach including prevention, risk factor management, wound care, reperfusion, and use of fibrinolytics and antioxidants. Patients with end stage renal disease represent the most affected population. This population often has multiple medications prescribed, some worth reconsidering before starting or continuing them. When possible, we recommend stopping all potentially contributing medications in patients with calciphylaxis, including warfarin, active vitamin D, calcium supplements, and iron.

Evaluating the validity of subclassifying warfarin-associated nonuremic calciphylaxis: a retrospective cohort study.

BACKGROUND: Calciphylaxis is a devastating multifactorial disorder of the subcutaneous fat that is known to be associated with hypercoagulability. Recent literature has proposed subclassifying patients with calciphylaxis as having warfarin-associated or warfarin-unassociated disease. AIM: We aimed to determine whether patients with warfarin-associated calciphylaxis differ clinically from patients with warfarin-unassociated calciphylaxis. MATERIALS AND METHODS: We performed a subgroup analysis of patients with nonuremic calciphylaxis from a previously studied cohort and compared clinical and outcomes features of patients who were taking warfarin at the time of disease onset to those of patients who were not. RESULTS: Nineteen patients with nonuremic calciphylaxis were identified, including 10 (53%) who had been on warfarin at the time of disease onset and 9 (47%) who had not. Of all clinical and outcomes parameters tested, no significant differences were detected between the two groups. DISCUSSION AND CONCLUSIONS: Though this study is limited by its retrospective nature and the relatively small number of patients studied, available data do not support subclassifying patients with nonuremic calciphylaxis as having warfarin-associated or warfarin-unassociated disease. Rather, the body of literature would suggest that identification and correction of underlying disorders of hypercoagulability should be prioritized.

Calcifilaxis no urémica mediada por acenocumarol / Non-uremic calciphylaxis due to acenocoumarol

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Tiosulfato sódico para el tratamiento de la calcifilaxia inducida por acenocumarol en un paciente sin enfermedad renal crónica / Sodium thiosulfate for the treatment of acenocumarol-induced calciphylaxis in a patient with preserved kidney function

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Acenocoumarol as a risk factor for calciphylaxis: a feature clinicians should be aware of.

In contrast with uraemic calciphylaxis in end-stage renal disease, causes of and risk factors for non-uraemic calciphylaxis are relatively unknown to clinicians and have yet to become fully established. This report describes a case of non-uraemic calciphylaxis, in which the use of acenocoumarol might have been a risk factor. It is important to raise awareness about this association among clinicians, as vitamin K antagonists have to be stopped for an optimal treatment of this severe condition.

Warfarin-Associated Nonuremic Calciphylaxis.

Importance: Classic calciphylaxis associated with renal failure is a life-threatening disease. Warfarin-associated calciphylaxis without renal injury has been described, but whether it is a subset of classic calciphylaxis or a different entity remains unknown. We describe 1 case of warfarin-associated calciphylaxis, present data from 2 others from our institution, and review all cases of warfarin-associated calciphylaxis available in the literature. Our review indicates that warfarin-associated calciphylaxis is clinically and pathophysiologically distinct from classic calciphylaxis. Objective: To review warfarin-associated calciphylaxis and determine its relationship to classic calciphylaxis. Design, Setting, and Participants: We searched MEDLINE and Ovid without language or date restrictions for case reports of calciphylaxis from the inpatient setting using the terms "calciphylaxis and warfarin," "non-uremic calciphylaxis," and "nonuremic calciphylaxis." We defined nonuremic calciphylaxis as a histopathologic diagnosis of calciphylaxis without severe kidney disease (serum creatinine level >3 mg/dL; glomerular filtration rate <15 mL/min; acute kidney injury requiring dialysis; and renal transplantation). Exposures: Each patient had been exposed to warfarin before the onset of calciphylaxis. Main Outcomes and Measures: Patient data were abstracted from published reports. Original patient medical records were requested and reviewed when possible. Results: We identified 18 patients with nonuremic calciphylaxis, 15 from the literature, and 3 from our institution. Patients were predominantly female (15 of 18 [83%]) with ages ranging from 19 to 86 years. Duration of warfarin therapy prior to calciphylaxis onset averaged 32 months. Lesions were usually located below the knees (in 12 of 18 [67%]). No cases reported elevated calcium-phosphate products (0 of 17 [0%]). Calcifications were most often noted in the tunica media (n = 8 [44%]) or in the vessel lumen and tunica intima (n = 7 [39%]). The most common treatments included substitution of heparin or low-molecular weight heparin for warfarin (n = 13 [72%]), intravenous sodium thiosulfate (n = 9 [50%]), and hyperbaric oxygen (n = 3 [17%]). The survival rate on hospital discharge was remarkably high, with 15 cases (83%) reporting full recovery and 3 cases ending in death. Conclusions and Relevance: Warfarin-associated calciphylaxis is distinct from classic calciphylaxis in pathogenesis, course, and, particularly, outcome. This finding should influence clinical management of the disease and informs targeted treatment of the disease.

Case Report of Calciphylaxis Secondary to Calcium and Vitamin D3 Supplementation.

BACKGROUND: Calciphylaxis is a rare disorder that is very unusual outside the setting of end-stage kidney disease. CASE SUMMARY: A 64-year-old woman with normal renal function presented with painful leg ulcers. She had previously received 300 000 IU of vitamin D3 followed by daily calcium and vitamin D3 supplementation. A skin biopsy was consistent with calciphylaxis, and she was treated with sodium thiosulphate infusions and wound debridement. CONCLUSION: Calcium and vitamin D3 supplements are widely prescribed. We report a case of calciphylaxis triggered by calcium and vitamin D3 supplementation in a patient with none of the typical risk factors. Our patient had an excellent response to treatment with sodium thiosulphate.