ABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Calciphylaxis/diagnosis , Calciphylaxis/therapy , Sepsis/prevention & control , Calciphylaxis/mortality , Diagnosis, Differential , Necrosis/complicationsABSTRACT
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.
Subject(s)
Humans , Female , Adult , Middle Aged , Calciphylaxis/diagnosis , Kidney Failure, Chronic/complications , Calciphylaxis/etiology , Calciphylaxis/therapy , Combined Modality Therapy , DebridementABSTRACT
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.