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Resumen Introducción : La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica me dia 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 ca racterísticas 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 di cumarínicos. 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.

Abstract Introduction : Calciphylaxis is a serious vascular dis order characterized by calcification of tunica media, in timal 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 stud ies 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, labo ratory 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. Sixty-one percent were men and 39% were NUC cases. Eighty-two 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 diagno sis 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 pa tients 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.

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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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Abstract The advanced stages of chronic kidney disease are associated with mineral and bone metabolism disorders, which increase the risk of serious complications such as uremic calciphylaxis. Below we present the case of a 65-year-old male patient with multiple comorbidities, including stage 5 chronic kidney disease with long-term hemodialysis treatment, who presented refractory secondary hyperparathyroidism complicated by penile necrosis secondary to uremic calciphylaxis. We believe this case may be useful in sensitizing the medical community on the seriousness of uremic calciphylaxis, emphasizing the importance of prevention and early diagnosis before complications such as necrosis occur. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).

Resumen Los estadios avanzados de la enfermedad renal crónica se asocian a alteraciones en el metabolismo mineral óseo, lo cual aumenta el riesgo de complicaciones graves como la calcifilaxis urémica. A continuación se presenta el caso de un paciente masculino de 65 años, con múltiples comorbilidades, entre ellas enfermedad renal crónica estadio 5 en terapia de hemodiálisis durante largo tiempo, quien presentó hiperparatiroidismo secundario de difícil manejo y se complicó con necrosis peneana secundaria a calcifilaxis urémica. Se considera que este caso puede ser útil para sensibilizar a la comunidad médica sobre la gravedad de la calcifilaxis urémica por lo cual es muy importante la prevención y realización de un diagnóstico temprano antes de que se produzcan complicaciones como la necrosis. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).

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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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Penile necrosis is an uncommon condition associated, in most cases, with calciphylaxis, also known as calcific uremic arteriolopathy. It is a progressive disease found in approximately 1%-4% of patients with end stage renal disease. Patients with penile calciphylaxis present a mortality rate of up to 70%, with life expectancy of two-and a half months following the diagnosis. We report a severe calciphylaxis case that had to be submitted to a penectomy but survived the penile event.

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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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Resumen La calcifilaxis es una enfermedad poco frecuente, aunque presenta una elevada tasa de mortalidad debido sobre todo a complicaciones como sepsis o gangrena. Generalmente se asocia a insuficiencia renal severa. Se define como la calcificación de la capa media de vasos de pequeño y mediano tamaño de la dermis y tejido celular subcutáneo. Clínicamente se manifiesta como un síndrome de livedoracemosa que progresa a púrpura retiforme y necrosis cutánea. La primera línea de tratamiento es el tiosulfato sódico.

Abstract Calciphylaxis is a rare disease, although it has a high mortality rate due mainly to complications such as sepsis or gangrene. It is usually associated with severe renal failure. It is defined as the calcification of the middle layer of small and medium-sized vessels of the dermis and subcutaneous cellular tissue. Clinically it manifests as a livedoracemosa syndrome that progresses to retinal purpura and cutaneous necrosis. The first line of treatment is sodium thiosulfate.

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Resumen La calcifilaxis es un síndrome caracterizado por la calcificación de la pared media de las arteriolas de pequeño y mediano calibre de dermis y tejido celular subcutáneo, que se caracteriza clínicamente por úlceras necróticas, intensamente dolorosas, que afectan sobre todo miembros inferiores. Existen factores de riesgo para su desarrollo, como insuficiencia renal crónica, hiperparatiroidismo, sexo femenino, raza caucásica, diabetes, uso de anticoagulantes, entre otros. Si bien no existe un protocolo estandarizado para su tratamiento, se ha propuesto el uso del tiosulfato sódico como primera elección. Se presenta el caso de una paciente de sexo femenino de 75 años de edad con diagnóstico de calcifilaxis.

Abstract Calciphylaxis is a syndrome characterized by calciphication of the media of small and medium vessels of dermis and subcutaneous celular tissue, clinically characterized by extremely painful, necrotic ulcers, that affects especially lower limbs. There are risk factors for its development, such as renal failure, hyperparathyroidism, female gender, caucasian race, diabetes, use of anticoagulants, among others. Although there is not a standarized protocol for the treatment, it has been proposed the use of sodium thiosulfate as first line. We report a case of a woman of 75 years old with calciphylaxis.

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Background: Penile necrosis is a rare condition that may present in patients with diabetes mellitus or chronic kidney disease (CKD). The recommended treatment is controversial. We report a case of penile necrosis in a diabetic patient caused by episode of paraphimosis associated with uremic arteriopathy treated with partial amputation. Clinical Case: A 53-year-old male with a background of arterial hypertension, diabetes mellitus and CKD in hemodialysis. The patient presented with paraphimosis and glans necrosis. An emergency circumcision was carried out. A doppler ultrasound found fluid collection in the left corpus cavernosum, parietal vascular calcifications and vascular insufficiency in the corpus cavernosum that suggested necrosis. A partial amputation of the penis was carried out. After three years of follow-up, the outcome has remained favorable. Conclusions: Penile necrosis is a rare but serious complication of terminal CKD. In these patients, systemic calciphylaxis is usually observed. The main take-away lesson is that a multidisciplinary approach is necessary.

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Resumen La calcifilaxis es una de las complicaciones menos comunes de la enfermedad renal crónica avanzada, sobretodo en terapia de sustitución renal, se desconoce la fisiopatología exacta de aparición, pero se cree, que es por una alteración en el metabolismo óseo-mineral. Se describe un caso clínico, de un paciente con enfermedad renal crónica, que presentó como complicación grave calcifilaxis, llegando a dicho diagnóstico gracias a las imágenes características de dicha patología tomadas del banco del servicio de imagenología del hospital. En conclusión, la calcifilaxis, a pesar de ser una patología difícil de encontrar en la actualidad, debido al mejor control del metabolismo óseo-mineral, se debe considerar en aquellos pacientes con progresión rápida de la enfermedad renal y con presencia de lesiones calcificadas supurativas en extremidades.

Abstract Calciphylaxis is one of the less common complications of Chronic Advanced Kidney Disease, especially in renal replacement therapy, the exact pathophysiology of its appearance is unknown, but it is believed that it is due to an alteration in bone-mineral metabolism. We describe a clinical case of a patient with chronic kidney disease, who presented as a serious complication calciphylaxis, reaching this diagnosis thanks to the characteristic images of this pathology taken from the bank of the Hospital's imaging service. In conclusion, calciphylaxis, despite being a pathology difficult to find nowadays due to better control of bone-mineral metabolism, should be considered especially in those patients with rapid progression of renal disease and presence of suppurative calcified lesions in extremities.

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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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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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A calcifilaxia é uma vasculopatia obliterativa caracterizada por necrose isquêmica cutânea, com instalação aguda e progressiva, secundária à calcificação de vasos sanguíneos de pequeno ou médio calibre. Habitualmente, é uma complicação observada nos pacientes com hiperparatireoidismo secundário à insuficiência renal crônica. Sua patogênese ainda é desconhecida e, provavelmente, multifatorial. Este estudo descreve o caso de um paciente com calcifilaxia idiopática submetido ao tratamento clínico, desbridamento cirúrgico e curativos, com resolução completa após 6 meses. Apesar de ser uma afecção rara e grave na população em geral, o diagnóstico e o tratamento precoce da calcifilaxia permitem a evolução clínica favorável e a melhoria da qualidade de vida do paciente.(AU)

The calciphylaxis is an obliterative vasculopathy characterized by cutaneous ischemic necrosis, with acute and progressive installation, secondary to calcification of small or medium-sized blood vessels. Usually, is a complication observed in patients with hyperparathyroidism secondary to chronic renal failure. Its pathogenesis is still unknown and probably multifactorial. This study describes a case of a patient with idiopathic calciphylaxis, which was submitted to clinical treatment, surgical debridement and dressings, with complete resolution after 6 months. Although being a rare and serious disease in the general population, the early diagnosis and treatment of calciphylaxis allow favorable clinical evolution and improvement of patient's quality of life.(AU)

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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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Objetivo: Revisar la literatura científica publicada en las principales bases de datos, entre los años 1962 y 2016, cuyo objetivo fue abordar el tema de la calcifilaxia y sus principales características. Metodología: Se llevó a cabo una revisión de la literatura en las principales bases de datos de las Ciencias de la Salud y las Ciencias Sociales (medline-pubmed, cinhal, web of science, lilacs, sociological abstracts, cuiden, embase, psycoinfo e isi web of knowledge). Se emplearon descriptores en español, inglés y portugués: Calcifilaxis, diálisis, hiperparatiroidismo, necrosis, gangrena, calcificación vascular, cuidados de enfermería y diagnósticos de enfermería. Se hizo una revisión sistemática de los artículos seleccionados utilizando un protocolo para extraer los datos. Resultado: Se encontraron 45 artículos que cumplían con los criterios de búsqueda establecidos, 35 reportaron los resultados de ensayos clínicos o estudios cualitativos, 4 correspondían a revisiones sistemáticas y 6 se catalogaron como estudios retrospectivos. Los autores señalaron las principales características de la enfermedad, de los pacientes y los tratamientos más usados, pero poco del cuidado que debe brindar el profesional enfermero. Conclusión: En los estudios analizados se identificaron principalmente aspectos sobre el curso de la enfermedad y el tratamiento; se reportó la efectividad de algunos tratamientos utilizadas en la atención de dichos pacientes, pero ninguna de ellas definitiva, lo que permite proponer algunas acciones desde la disciplina enfermera.

Objective: To analyze the scientifi c literature published in the main databases between 1962 and 2016, whose objective was to address the issue of calciphylaxis and its main characteristics? Methodology: A literature review was carried out in the main databases of the Health Sciences and Social Sciences (medline-pubmed, cinhal, web of science, lilacs, sociological abstracts, cuiden, embase, psycoinfo and isi web of knowledge). An analytical review of the selected articles was made using a protocol to extract the data. Results: 45 articles were found that fulfi lled the established search criteria, 35 reported the results of clinical trials or qualitative studies, 4 corresponded to systematic reviews and 6 were classifi ed as retrospective studies. The authors pointed out the main characteristics of the disease, the patients and the most used treatments, but little of the care that should be provided by the nurse practitioner. Conclusion: In the analyzed studies, aspects regarding the course of the disease and treatment were identifi ed; it was reported the effectiveness of some treatments used in the care of these patients, but none of them defi nitive, which allows to propose some actions from the nurse discipline.

Objetivo: Revisar a literatura científica publicada nas principais bases de dados, entre os anos 1962 e 2016, cujo objetivo era abordar o tema da calcifilaxia e suas principais características. Metodologia: Uma revisão da literatura foi realizada nas principais bases de dados das Ciências da Saúde e das Ciências Sociais (Medline-Pubmed, Cinhal, Web of Science, Lilacs, Sociological abstracts, Cuiden, Embase, Psycoinfo e Isi Web of Knowledge). Foram usados descritores em espanhol, inglês e português: calcifilaxia, diálise, hiperparatiroidismo, necrose, gangrena, calcificação vascular, cuidados de enfermagem e diagnósticos de enfermagem. Foi feita uma revisão sistemática dos artigos selecionados utilizando um protocolo para extrair os dados. Resultados: Encontraram-se 45 artigos que cumpriam com os critérios de busca estabelecidos, 35 reportaram os resultados de ensaios clínicos ou estudos qualitativos, quatro (4) correspondiam a revisões sistemáticas e seis (6) se catalogaram como estudos retrospectivos. Os autores apontaram as principais características da doença, dos pacientes e os tratamentos mais usados, mas pouco do cuidado que debe oferecer o professional da enfermagem. Conclusão: Nos estudos analisados se identificaram principalmente aspectos sobre o curso da doença e o tratamento; se reportou a efetividade de alguns tratamentos utilizados no atendimento desses pacientes, mas nenhum deles definitivo, o que permite propor algumas ações desde a profissão da enfermagem.

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Calciphylaxis is vasculopathy characterized by ischemia and painful skin necrosis due to calcification and intimal fibroplasia of thrombosis of the panicular arterioles. It most frequently compromises patients with terminal chronic renal failure and has a high mortality rate. Biopsy of skin lesions is used as a diagnostic method. No specific laboratory findings have been recorded. Skin lesions usually begin in the extremities like a painful purplish mottling similar to "livedo reticularis". The natural evolution is to ulcers and bedsores. The first line of treatment involves the care of skin lesions and antibiotic therapy. Sodium thiosulfate is used as treatment due to its antioxidant activity and as a chelating. Two clinical cases are here reported.

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RESUMO

La calcifilaxis es una vasculopatía caracterizada por isquemia y necrosis cutánea dolorosa debida a calcificación, fibroplastia de la íntima y trombosis de las arteriolas paniculares. Compromete más frecuentemente a pacientes con insuficiencia renal crónica terminal y tiene muy elevada mortalidad. La biopsia de las lesiones cutáneas se utiliza como método diagnóstico. No se han registrado hallazgos específicos de laboratorio. Las lesiones cutáneas generalmente comienzan en las extremidades a modo de moteado violáceo doloroso similar al livedo reticularis. La evolución natural es hacia úlceras y escaras. La primera línea de tratamiento consiste en el cuidado de las lesiones cutáneas y antibioticoterapia. El tiosulfato sódico se utiliza como tratamiento debido a su actividad como antioxidante y quelante. Se presentan dos casos clínicos.

Calciphylaxis is vasculopathy characterized by ischemia and painful skin necrosis due to calcification and intimal fibroplasia of thrombosis of the panicular arterioles. It most frequently compromises patients with terminal chronic renal failure and has a high mortality rate. Biopsy of skin lesions is used as a diagnostic method. No specific laboratory findings have been recorded. Skin lesions usually begin in the extremities like a painful purplish mottling similar to "livedo reticularis". The natural evolution is to ulcers and bedsores. The first line of treatment involves the care of skin lesions and antibiotic therapy. Sodium thiosulfate is used as treatment due to its antioxidant activity and as a chelating. Two clinical cases are here reported.

Assuntos