Calciphylaxis, a diagnostic and therapeutic challenge: report of a successful case

Abstract

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. Keywords: Blood vessels; Calcium metabolism disorders; Hyperparathyroidism, secondary; Ischemia; Renal insufficiency, chronic Resumo: 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.

A Fatal Case of Non-Uremic Calciphylaxis: A Case Report and Literature Review

“…Calciphylaxis is a rare cutaneous disease, also known as calcific uremic arteriolopathy (CUA), that occurs most frequently in patients with advanced chronic kidney disease and on long-term hemodialysis [ 1 ]. It is a rare condition, described in 1% to 4% of patients on dialysis, mainly in those with a history of diabetes mellitus (DM), liver disease, and calcium-phosphate product level of more than 70 mg 2 /dL 2 [ 2 ].…”

Calciphylaxis: a rare but potentially fatal event of chronic kidney disease. Case report

“…Its synonym "calcific uremic arteriolopathy" is an adequate descriptive term, even though the disease can occur in patients with normal renal function. 3 Calciphylaxis has been reported to occur in 1% to 4.5% of patients in dialysis, mostly in hemodialysis, with preponderance in patients who are obese, diabetic, present liver disease, are using systemic corticosteroids or have a calcium-phosphate product of more than 70mg 2 /dL 2 . 1,3 The classical clinical picture is that of an initial skin lesion, livedo reticularis-like on the lower limbs, which progress to violaceous, painful, plaque or subcutaneous nodules, followed by ischemic/necrotic ulcers of reticular pattern.…”

“…3 Calciphylaxis has been reported to occur in 1% to 4.5% of patients in dialysis, mostly in hemodialysis, with preponderance in patients who are obese, diabetic, present liver disease, are using systemic corticosteroids or have a calcium-phosphate product of more than 70mg 2 /dL 2 . 1,3 The classical clinical picture is that of an initial skin lesion, livedo reticularis-like on the lower limbs, which progress to violaceous, painful, plaque or subcutaneous nodules, followed by ischemic/necrotic ulcers of reticular pattern. 4,5 Usually, the patients present hyperphosphatemia, hyperparathyroidism and an elevated plasma calcium and phosphate product.…”

A case of calciphylaxis with an unfavorable outcome

“… 1 It is observed in 1% to 4.5% of dialysis patients, especially those undergoing hemodialysis and women. 2 Hyperparathyroidism (HPT) secondary to nephropathy leads to changes in the metabolism of calcium (Ca) and phosphorus (P), representing the main etiological factor.…”

“…Its etiopathogenesis is not fully established and there is calcification and obstruction of the small and medium caliber blood vessels of the dermis, intima and media hyperplasia, and septal and/or lobular subcutaneous necrosis leading to distal ischemia and necrotic ulcers. 2 Systemic ischemic impairment, such as acute myocardial infarction, stroke, mesenteric ischemia, and peripheral arterial obstructions, may also occur. 4 …”