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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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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.

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La calcifilaxis o arteriolopatía urémica calcificante es una enfermedad rara que conlleva elevada morbilidad y una mortalidad de 40-80%. Se produce por la calcificación de los vasos de pequeño calibre y afecta sobre todo a los pacientes con insuficiencia renal crónica. Suele iniciarse con cambios en la coloración de la piel. Luego se produce ulceración, dolor y necrosis cutánea. Se presenta el caso de un varón de 70 años con insuficiencia renal crónica que desarrolló lesiones cutáneas dolorosas en los miembros inferiores.

Calciphylaxis or calcifying uremic arteriolopathy is a rare disease that carries high morbidity and mortality between 40% and 80%. It is produced by calcification of small caliber vessels and mainly affects patients with chronic renal failure. It usually begins with a change in skin color and then ulceration, pain and skin necrosis occur. We present a 70-year-old man with chronic renal failure who developed painful skin lesions on his lower limbs.

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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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A 66-year-old male presented with five days of penile pain and ulceration. The patient had a history of stage 5 chronic kidney disease and repeatedly declined hemodialysis. Wound and urine cultures were unrevealing. CT of the abdomen and pelvis did not reveal any evidence of Fournier's gangrene but identified diffuse severe calcific vasculopathy. Urology and dermatology agreed on the diagnosis of penile calciphylaxis. While diagnosis of calciphylaxis often includes histologic evidence of obstructive vasculopathy, biopsy of penile calciphylaxis is contraindicated due to increased morbidity and mortality. Management focuses wound care and correction of electrolyte abnormalities responsible for calcium deposition.

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ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

RESUMO Introdução: O impacto clínico da calcificação vascular está bem estabelecido no âmbito de morbimortalidade cardiovascular, mas outras síndromes clínicas, como a calcifilaxia, apesar de menos frequente, têm significante impacto na doença renal crônica. Métodos: Relato de caso de mulher, 27 anos, com queixa de dor em pododáctilos bilateralmente havia 3 dias, com presença de pequenas áreas necróticas nos locais referidos. Antecedente pessoal de diabetes tipo 1 (há 25 anos), com doença renal crônica, em diálise peritoneal, além de artrite reumatoide. Teve internação hospitalar, que antecedeu o quadro atual, devido à exacerbação da artrite reumatoide, evoluindo com trombo intracardíaco por complicação de cateter venoso, quando iniciou uso de varfarina. A isquemia progrediu para pés com necessidade de amputações bilaterais. Quirodáctilos também foram acometidos. Trombofilias, vasculites, endocardite ou outras fontes emboligênicas foram pesquisadas e descartadas. Anatomopatológico evidenciou: necrose de pele e partes moles superficiais com trombose arterial recente e calcificação medial de Monckeberg. Tratamento foi instituído com bisfosfonato e tiossulfato de sódio, conversão para hemodiálise e substituição de varfarina por heparina não fracionada. Apesar de toda a terapia, a paciente foi a óbito após quatro meses de evolução. Discussão: A calcifilaxia é uma rara síndrome de calcificação da microvasculatura que resulta em graves lesões isquêmicas. Tem patogênese relacionada ao distúrbio mineral e ósseo da doença renal crônica combinado com o desbalanço entre promotores e inibidores de calcificação vascular, com particular importância ao antagonismo da vitamina K. Conclusão: A estratégia preventiva é fundamental, uma vez que a terapia é complexa e de eficácia pouco validada.

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INTRODUCTION: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. METHODS: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. DISCUSSION: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. CONCLUSION: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

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Antecedentes: La arteriolopatia calcificante urémica o calcifilaxis es un síndrome raro, potencialmente mortal, que afec-ta casi en exclusiva a pacientes con insuficiencia renal y diálisis, caracterizado por calcificación vascular de arterias de pequeño y mediano calibre, con posterior proliferación, fibrosis y trombosis que conducen finalmente a necrosis y úlceras cutáneas. Se asocia con la enfermedad renal crónica terminal y trasplante renal, con preva-lencia de 1-4% de los pacientes con insuficiencia renal crónica. El tratamiento es especializado a base de cámara hiperbárica y para-tiroidectomía para inducir curación. Descripción del caso clínico: Femenina de 42 años, captada en la consulta externa de nefrología en el Instituto Hondureño de Seguridad Social en el año 2017, con antecedente de hipertensión arterial y nefropatía crónica, sometida a trasplante renal en 1998 el cual fue fallido, posteriormente en pro-grama de hemodiálisis y manejo conservador desde el año 2005. La paciente desarrolló lesiones equimóticas en tronco y úlceras en sitios de nódulos subcutáneos que se sobreinfectaron, desarrollan-do signos de respuesta inflamatoria sistémica. Los exámenes de laboratorio mostraron hiperfosfatemia, paratohormona 3518 pg/ml, producto calcio-fósforo 73.5. Ante la falta de manejo quirúrgico (pa-ratiroidectomía) y cámara hiperbárica en la institución, en el 2017 se estableció tratamiento conservador a base de antibióticos, analgési-cos, y hemodiálisis diarias, con lo que presentó mejoría del cuadro clínico; sin embargo, sin resolución de su cuadro de base de la calci-filaxis. Conclusión: El manejo conservador en el caso de pacientes con calcifilaxis es una opción de tratamiento disponible con buena respuesta en pacientes con seguimiento estrecho...(AU)

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