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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.

Introducción: La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica media arteriolar, trombosis y necrosis cutánea. Se describió en pacientes con insuficiencia renal (CU), aunque puede producirse en su ausencia (CNU). Sus factores de riesgo están en estudio y su diagnóstico puede ser complejo. Su mortalidad se estimaba en 60-80%, aunque trabajos recientes indican que ha disminuido (40%). Métodos: Estudio retrospectivo entre el 1/1/2011 y el 31/12/2019. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, y la evolución de todos los pacientes con diagnóstico de calcifilaxis evaluados en el Hospital Italiano de Buenos Aires. Resultados: Se incluyeron 39 pacientes. El 61.5% (24) eran hombres y 38.5% (15) fueron casos de CNU. De éstos, 82% presentaba hipertensión arterial, 66% obesidad y 46% diabetes. El 49% recibía anticoagulantes dicumarínicos. Todos los pacientes con CNU y 75% con CU presentaron úlceras de fondo necrótico, localizadas con mayor frecuencia en las piernas. En 72% de los casos el diagnóstico histológico se efectuó con una toma de biopsia. En todos, el tratamiento fue multimodal y la mortalidad al año fue de 42%. Conclusión: Observamos una elevada proporción de pacientes con CNU, en relación con lo comunicado en la literatura, y la mitad recibía anticoagulantes dicumarínicos. El diagnóstico histológico se efectuó por biopsia en la mayor parte de los casos, para lo cual la toma quirúrgica de la muestra, la tinción con Von Kossa y la evaluación por un patólogo experto fueron claves.

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La calcifilaxis se caracteriza por una intensa deposición de calcio en pequeños vasos sanguíneos, piel y otros órganos, descripta principalmente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción paratiroidea. Hasta la fecha, solo hay siete casos descriptos en la literatura de calcifilaxis que imita arteritis de células gigantes (ACG). En esta revisión presentamos el octavo caso documentado patológicamente.

Calciphylaxis is characterized by intense deposition of calcium in small blood vessels, skin, and other organs, described mainly in patients with chronic renal insufficiency, renal transplant of parathyroid dysfunction. To date, there are only seven cases described in literature of calciphylaxis mimicking giant cell arteritis (GCA). In this review, we present the eighth case pathologically documented.

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Abstract Introduction: Calciphylaxis is an infrequent disease that almost exclusively affects patients with chronic kidney disease, although cases have been observed in patients without renal function impairment. The diagnosis is mainly made by clinical manifestations and subsequently confirmed by radiological and histological study. The optimal treatment is not known, although there is a consensus that a multifactorial approach is required. Clinical Case: A 68-year-old woman on hemodialysis for 2 years, who presented a painful nodular lesion in the left thigh, a skin biopsy was performed resulting in a diagnosis of calciphylaxis. Treatment and Outcome: Treatment was started with intravenous sodium thiosulfate. Pamidronate is added intravenously, three months later, due to an unfavorable evolution. After 6 months of treatment, improvement in nodular lesions and healing of the ulcerated lesion was observed to be generally well tolerated treatment. Conclusion: The combined treatment of sodium thiosulfate, pamidronate and calcitomimetics has been effectiveand safe for the treatment of calciphylaxis, inducing complete remission.

Resumen: Introducción: La calcifilaxis es una enfermedad infrecuente que afecta casi exclusivamente a pacientes con insuficiencia renal, aunque se han observado casos en pacientes sin deterioro de la función renal. El diagnóstico es clínico confirmándose con estudio radiológico e histológico. No se conoce con exactitud el tratamiento óptimo, aunque hay consenso en que se requiere un abordaje multifactorial. Caso Clínico: Mujer de 68 años en hemodiálisis desde hace 2 años, que presenta una lesión nodular dolorosa en muslo izquierdo, resultando un diagnostico compatible con calcifilaxis, tras biopsia cutánea. Tratamiento y resultado: Inicia tratamiento con tiosulfato de sodio vía venosa. Tres meses más tarde y ante la evolución desfavorable, se añade al tratamiento pamidronato vía intravenosa. Tras 6 meses de tratamiento se observa mejoría de las lesiones nodulares y cicatrización de la lesión ulcerada, habiéndose experimentado buena tolerancia. Conclusión: El tratamiento combinado de tiosulfato de sodio, pamidronato y calcimiméticos ha resultado efectivo y seguro para el tratamiento de la calcifilaxis, induciendo su remisión completa.

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Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

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Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

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Calciphylaxis, also known as calcific uremic arteriolopathy and uremic small artery disease with medial wall calcification and intimal hyperplasia, is a multifactorial cutaneous vascular disease characterized by chronic, painful, non-healing wounds that occur frequently in patients with chronic kidney disease, predominantly in those with end-stage renal disease. The pathogenesis remains unclear, and the development of calciphylaxis lesions depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. Despite an increase in reports of calciphylaxis in the literature and clinical recognition of demographic characteristics and risk factors associated with calciphylaxis, it remains a poorly understood disease with high morbidity and mortality. In this review, we analyze and summarize the clinical manifestations, pathogenesis and pathophysiology, histopathology, differential diagnosis, diagnostic workup and treatment modalities for calciphylaxis. Because of the lack of consensus regarding the optimal approach to and treatment of this disorder, a high degree of clinical suspicion, early diagnosis, and multimodal and multidisciplinary treatment in collaboration with dermatology, nephrology, wound care, nutrition and pain management specialties may improve survival in patients with calciphylaxis.

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INTRODUCTION: Calciphylaxis is an infrequent disease that almost exclusively affects patients with chronic kidney disease, although cases have been observed in patients without renal function impairment. The diagnosis is mainly made by clinical manifestations and subsequently confirmed by radiological and histological study. The optimal treatment is not known, although there is a consensus that a multifactorial approach is required. CLINICAL CASE: A 68-year-old woman on hemodialysis for 2 years, who presented a painful nodular lesion in the left thigh, a skin biopsy was performed resulting in a diagnosis of calciphylaxis. TREATMENT AND OUTCOME: Treatment was started with intravenous sodium thiosulfate. Pamidronate is added intravenously, three months later, due to an unfavorable evolution. After 6 months of treatment, improvement in nodular lesions and healing of the ulcerated lesion was observed to be generally well tolerated treatment. CONCLUSION: The combined treatment of sodium thiosulfate, pamidronate and calcitomimetics has been effectiveand safe for the treatment of calciphylaxis, inducing complete remission.

INTRODUCCIÓN: La calcifilaxis es una enfermedad infrecuente que afecta casi exclusivamente a pacientes con insuficiencia renal, aunque se han observado casos en pacientes sin deterioro de la función renal. El diagnóstico es clínico confirmándose con estudio radiológico e histológico. No se conoce con exactitud el tratamiento óptimo, aunque hay consenso en que se requiere un abordaje multifactorial. CASO CLÍNICO: Mujer de 68 años en hemodiálisis desde hace 2 años, que presenta una lesión nodular dolorosa en muslo izquierdo, resultando un diagnostico compatible con calcifilaxis, tras biopsia cutánea. TRATAMIENTO Y RESULTADO: Inicia tratamiento con tiosulfato de sodio vía venosa. Tres meses más tarde y ante la evolución desfavorable, se añade al tratamiento pamidronato vía intravenosa. Tras 6 meses de tratamiento se observa mejoría de las lesiones nodulares y cicatrización de la lesión ulcerada, habiéndose experimentado buena tolerancia. CONCLUSIÓN: El tratamiento combinado de tiosulfato de sodio, pamidronato y calcimiméticos ha resultado efectivo y seguro para el tratamiento de la calcifilaxis, induciendo su remisión completa.

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An 82-year-old woman came to consultation with sudden visual loss in her left eye. Fifteen days before, she complained of diplopia. She had doubtful symptoms of giant cell arteritis and showed a normal physical exam. Lab results showed erythrosedimentation rate (ESR) = 62 mm/1°h; uremia = 0.56 g/dl (normal <0.45); serum creatinine = 1.7 mg% (normal <1.4); low calcium and phosphorus; and normal urine calcium and serous PTH. Fundus exam and brain magnetic resonance imaging (MRI) showed normality of optic nerves, chiasma, retrochiasmatic area, ocular muscles, eyeballs, lacrimal glands, periorbital fat, cavernous sinuses, and occipital cortex. A temporal arteritis was suspected; therefore, a biopsy was carried on. It showed the presence of large calcium deposits in the artery's tunica media and internal elastic lamina, with narrowing of the lumen, with no inflammation and multinuclear giant cells. Histological diagnosis is calciphylaxis. Although calciphylaxis is a well-described entity that occurs in end-stage renal patients, many cases are due to non-uremic causes. To date, there are only six cases described in literature of calciphylaxis mimicking GCA.

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Calciphylaxis or calcific uremic arteriolopathy is a rare cutaneous-systemic disease occurring in patients with advanced chronic kidney disease. The classical clinical picture is that of a necrotic and progressive skin ulcer of reticular pattern, mostly in the lower legs and susceptible to local infection. It is a product of mural calcification and occlusion of cutaneous and sub-cutaneous arteries and arterioles. The authors report the case of a 73-year-old male patient in his late stage of renal disease presenting severe necrotic cutaneous ulcers on lower legs followed by local and systemic infection and death due to sepse after parathyroidectomy.

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Calciphylaxis or calcific uremic arteriolopathy is a rare cutaneous-systemic disease occurring in patients with advanced chronic kidney disease. The classical clinical picture is that of a necrotic and progressive skin ulcer of reticular pattern, mostly in the lower legs and susceptible to local infection. It is a product of mural calcification and occlusion of cutaneous and sub-cutaneous arteries and arterioles. The authors report the case of a 73-year-old male patient in his late stage of renal disease presenting severe necrotic cutaneous ulcers on lower legs followed by local and systemic infection and death due to sepse after parathyroidectomy.

Calcifilaxia ou arteriolopatia urêmica calcificante é quadro cutâneo-sistêmico raro em paciente com doença renal crônica em fase de diálise. Caracteriza-se por úlcera cutânea, necrose de padrão retiforme, evolução progressiva, localizada principalmente nos membros inferiores e suscetibilidade à infecção secundária. Decorre de calcificação da parede arterial e oclusão de arteríolas e artérias cutâneas e subcutâneas. Os autores relatam caso de paciente do sexo masculino de 73 anos de idade com doença renal em estadio tardio e úlceras cutâneas nos membros inferiores com necrose, evolução grave, infecção local e sistêmica e, óbito por sepse após paratireoidectomia.

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AIM: The purpose of this study was to identify placental and umbilical cord macroscopic changes and correlate them to maternal and fetal clinical events in hypertensive disorders of pregnancy (HDP). MATERIALS AND METHODS: The authors examined 150 placentas, 30 from each HDP group, totaling 120, and 30 from the control group. All placentas and umbilical cords were examined, recorded, and photographed. RESULTS: The mean placental weight in the control group (526.3 +/- 95.6 g) was greater than in the HDP (435.5 +/- 43.1 g). Calciphylaxis was the most common macroscopic change found in the control and HDP groups in 27 (90%) and 118 cases (98.3%), respectively. DISCUSSION: Pregnant women with HDP were relatively younger. In addition, due to low blood flow seen in HDP, the macroscopic changes found included lower placental weight, calciphylaxis in the maternal surface, and fibrin in the fetal surface. Because of all complications associated, most women with HDP had preterm infants who developed respiratory problems and had shorter umbilical cords.

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Calciphylaxis is an infrequent but severe entity found in chronic dialysis patients. Its clinical pattern consists of tissue ischemia with itchy and painful subcutaneous nodules and plaques, most often located on the abdomen, buttocks, thighs and/or legs. These injuries evolve to extensive superficial necrosis of the skin overlying the panniculitis, with ulceration, overinfection and consequent sepsis. Current treatment modalities used to counteract this pathology are not entirely effective. A new treatment reported for calciphylaxis, is the use of intravenous sodium thiosulfate. This inorganic salt is already used in the treatment of intoxication caused by cyanide, in patients with calcific nephrolithiasis and tumoral calcinosis, with very good and safe results. We herewith report a case of calciphylaxis that was cured using intravenous sodium thiosulphate treatment.

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BACKGROUND: Calciphylaxis is a serious condition characterized by ischemic ulceration of the skin and necrosis secondary to dystrophic calcification of the subcutaneous tissue and small arteries. It affects primarily patients with end-stage renal disease with prevalence up to 4%. However, penile calciphylaxis has been reported in only 37 cases in the international literature. We report one case, to review the literature and to provide the basis for a rational treatment of calciphylaxis of the penis that reduces the associated mortality. CASE REPORT: We report the case of an 82-year-old male with a 15-year history of type 2 diabetes mellitus and renal insufficiency during the past 2 years. He presented an increase of consistency of the distal portion in the penis' glans and scrotal swelling, evolving to complete penile glans necrosis. CONCLUSIONS: With the increase of the number of patients on dialysis treatment, the prevalence of calciphylaxis will increase. A high index of suspicion is warranted in all patients with end-stage renal disease and who present the characteristic genital lesions. Treatment of this complication must include avoidance of all exogenous calcium, administration of calcitriol analogues to offset the PTH, emergency parathyroidectomy in the case of overt hyperparathyroidism and aggressive surgical treatment with total or partial penectomy.

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BACKGROUND: Calcific uremic arteriolopathy is a rare and serious disorder characterized by systemic medial calcification of the arteries and tissue ischemia. Most often it is found in patients with chronic renal failure on dialysis and in renal transplant recipients with secondary hyperparathyroidism. METHODS: We report six patients with end-stage renal disease [five on hemodialysis (one with a nonfunctioning renal graft) and one on peritoneal dialysis] who developed painful livedo reticularis and skin necrosis of the limbs. All had secondary hyperparathyroidism and elevated calcium-phosphorus product. Our patients presented with the following clinical features: white race (six patients), hypoalbuminemia (three patients), diabetes (one patient), and obesity (four patients). RESULTS: Subtotal parathyroidectomy was performed in three cases. Despite this procedure, two patients died; one patient survived and his lesions healed. One patient was treated with aggressive wound care and hemodialysis (with low dialysate calcium concentration and Renagel phosphorus binders) and one patient received only local wound care, both with improvement of their lesions. In one case, no therapy was performed because the patient died immediately after diagnosis. CONCLUSIONS: The three patients who survived (Cases 4, 5 and 6) had distal lesions, normal serum albumin, and an early diagnosis. There was a relationship between the outcome of the patients and these factors, rather than the type of treatment received.

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