RESUMEN
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.
Asunto(s)
Calcifilaxia , Humanos , Estudios Retrospectivos , Masculino , Femenino , Factores de Riesgo , Persona de Mediana Edad , Calcifilaxia/terapia , Calcifilaxia/patología , Calcifilaxia/mortalidad , Calcifilaxia/diagnóstico , Anciano , Adulto , Anciano de 80 o más Años , Anticoagulantes/uso terapéutico , Resultado del Tratamiento , Argentina/epidemiologíaRESUMEN
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.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Calcifilaxia/diagnóstico , Calcifilaxia/terapia , Sepsis/prevención & control , Calcifilaxia/mortalidad , Diagnóstico Diferencial , Necrosis/complicacionesRESUMEN
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.
Asunto(s)
Calcifilaxia/terapia , Calcifilaxia/diagnóstico , Calcifilaxia/etiología , Calcifilaxia/patología , Humanos , Piel/patologíaRESUMEN
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.
Asunto(s)
Calcifilaxia/diagnóstico , Fallo Renal Crónico/complicaciones , Adulto , Calcifilaxia/etiología , Calcifilaxia/terapia , Terapia Combinada , Desbridamiento , Femenino , Humanos , Persona de Mediana EdadRESUMEN
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.
Asunto(s)
Humanos , Femenino , Adulto , Persona de Mediana Edad , Calcifilaxia/diagnóstico , Fallo Renal Crónico/complicaciones , Calcifilaxia/etiología , Calcifilaxia/terapia , Terapia Combinada , DesbridamientoRESUMEN
Skin manifestations associated with chronic kidney disease are very common. Most of these conditions present in the end stages and may affect the patient's quality of life. Knowledge of these entities can contribute to establishing an accurate diagnosis and prognosis. Severe renal pruritus is associated with increased mortality and a poor prognosis. Nail exploration can provide clues about albumin and urea levels. Nephrogenic systemic fibrosis is a preventable disease associated with gadolinium contrast. Comorbidities, such as diabetes mellitus and secondary hyperparathyroidism, can lead to acquired perforating dermatosis and calciphylaxis, respectively. Effective and innovative treatments are available for all of these conditions.
Asunto(s)
Insuficiencia Renal Crónica/patología , Enfermedades de la Piel/etiología , Piel/patología , Calcifilaxia/etiología , Calcifilaxia/terapia , Comorbilidad , Enfermedades del Cabello/etiología , Enfermedades del Cabello/patología , Enfermedades del Cabello/terapia , Humanos , Enfermedades de la Uña/etiología , Enfermedades de la Uña/patología , Enfermedades de la Uña/terapia , Dermopatía Fibrosante Nefrogénica/etiología , Dermopatía Fibrosante Nefrogénica/terapia , Porfiria Cutánea Tardía/etiología , Porfiria Cutánea Tardía/terapia , Pronóstico , Prurito/etiología , Prurito/terapia , Insuficiencia Renal Crónica/complicaciones , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/patología , Enfermedades de la Piel/terapia , Terapias en InvestigaciónRESUMEN
BACKGROUND: Calciphylaxis is a rare and severe disease with an annual incidence of around 1 % in dialysis patients. The main study aim was to determine its incidence in Martinique, where there is a significant population of patients on dialysis. PATIENTS AND METHODS: All patients diagnosed with calciphylaxis between 2006 and 2012 and living in Martinique were included, retrospectively. Social, demographic, biological, anatomic, pathological, histological and outcome data were analysed. RESULTS: Fifteen patients were included (8 women, 7 men). The incidence of calciphylaxis in this population was about 4.62/1,000,000 inhabitants per year. All patients presented very painful skin ulcerations and necrosis, chiefly on the lower extremities in 53.3 % of cases. All patients were on haemodialysis and two had undergone renal transplantation. Fourteen of the 15 patients were presenting secondary hyperparathyroidism, 12 had hypertension, 9 peripheral arterial disease, 8 obesity and 8 diabetes mellitus. Raised calcium and phosphorus were noted in 8 patients, with hypoalbuminaemia in 9 patients. Treatment with sodium thiosulfate was given for 8 patients, and was beneficial for all after a mean duration of 3.4 months. After 6 months of follow-up, 8 of the 15 patients were cured, 1 showed improvement and 6 had died. CONCLUSION: To our knowledge, this is the first study to examine the incidence of calciphylaxis in the general population. The relatively large number of patients could be accounted for by the high number of comorbidities in end-stage renal disease patients in Martinique, including obesity, diabetes, hypertension and arteritis. Treatment with sodium thiosulfate was beneficial for 8 patients.
Asunto(s)
Calcifilaxia/epidemiología , Amputación Quirúrgica , Calcifilaxia/etiología , Calcifilaxia/terapia , Femenino , Humanos , Hiperparatiroidismo Secundario/complicaciones , Incidencia , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/cirugía , Fallo Renal Crónico/terapia , Trasplante de Riñón , Úlcera de la Pierna/etiología , Masculino , Martinica/epidemiología , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Pronóstico , Diálisis Renal , Estudios Retrospectivos , Tiosulfatos/uso terapéuticoRESUMEN
Calciphylaxis, also known as calcific uremic arteriolopathy, is a severe complication often observed in patients with hyperparathyroidism secondary to chronic renal failure, which occurs mainly in women. It is characterized by ischemic tissue loss secondary to progressive vascular compromise. This is a rare and severe condition and its pathogenesis is unclear. The best treatment is prevention, especially in order to maintain adequate levels of calcium and phosphorus. We describe a case of this disease in a patient with hyperparathyroidism secondary to chronic renal failure who underwent medical treatment, surgical debridement and total skin autografts, with resolution of symptoms after 6 months.
Asunto(s)
Calcifilaxia/etiología , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/complicaciones , Calcifilaxia/diagnóstico , Calcifilaxia/terapia , Femenino , Humanos , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
Calciphylaxis, also known as calcific uremic arteriolopathy, is a severe complication often observed in patients with hyperparathyroidism secondary to chronic renal failure, which occurs mainly in women. It is characterized by ischemic tissue loss secondary to progressive vascular compromise. This is a rare and severe condition and its pathogenesis is unclear. The best treatment is prevention, especially in order to maintain adequate levels of calcium and phosphorus. We describe a case of this disease in a patient with hyperparathyroidism secondary to chronic renal failure who underwent medical treatment, surgical debridement and total skin autografts, with resolution of symptoms after 6 months.
A Calcifilaxia, também conhecida como arteriolopatia urêmica calcificante, é uma complicação freqüentemente observada nos pacientes com hiperparatireoidismo secundário à insuficiência renal crônica, ocorrendo principalmente em mulheres. Caracteriza-se por necrose isquêmica cutânea, de instalação aguda e progressiva, secundária à calcificação de vasos sanguíneos de pequeno ou de médio calibre. Trata-se de uma afecção rara e grave, cuja patogênese é obscura, e o melhor tratamento é a prevenção, especialmente visando manter os níveis adequados de cálcio e de fósforo. Descrevese um caso desta moléstia em paciente portadora de hiperparatireoidismo secundário à insuficiência renal crônica, submetida a tratamento clínico, desbridamento cirúrgico e enxertos autólogos de pele total, com resolução completa do quadro após 6 meses.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Calcifilaxia/etiología , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/complicaciones , Calcifilaxia/diagnóstico , Calcifilaxia/terapia , Resultado del TratamientoRESUMEN
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.