Calciphylaxis: a rare cause of limb ulcer – a case report

BACKGROUND: Calciphylaxis is a rare disease with a poor prognosis predominantly found in end-stage renal disease patients treated with dialysis. This pathology most commonly presents with painful non-healing ulcers, predisposing to high risk of sepsis and death. The authors present a case of bilate...

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Main Authors: Roberto Cunha, Manuel Silva, Vítor Carneiro, Pedro Sousa, Mafalda Correia, Isabel Vieira, Isabel Cássio, Nelson Oliveira, Emanuel Dias
Format: Article
Language:Portuguese
Published: Sociedade Portuguesa de Angiologia e Cirurgia Vascular 2024-03-01
Series:Angiologia e Cirurgia Vascular
Subjects:
Online Access:https://acvjournal.com/index.php/acv/article/view/546
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author Roberto Cunha
Manuel Silva
Vítor Carneiro
Pedro Sousa
Mafalda Correia
Isabel Vieira
Isabel Cássio
Nelson Oliveira
Emanuel Dias
author_facet Roberto Cunha
Manuel Silva
Vítor Carneiro
Pedro Sousa
Mafalda Correia
Isabel Vieira
Isabel Cássio
Nelson Oliveira
Emanuel Dias
author_sort Roberto Cunha
collection DOAJ
description BACKGROUND: Calciphylaxis is a rare disease with a poor prognosis predominantly found in end-stage renal disease patients treated with dialysis. This pathology most commonly presents with painful non-healing ulcers, predisposing to high risk of sepsis and death. The authors present a case of bilateral leg ulcers due to calciphylaxis. CASE REPORT: A 40-year-old female patient presented with painful ulcers in both lower limbs, for two months. She had a history of end-stage renal disease, morbid obesity, severe hypertension, arterial fibrillation and secondary hyperparathyroidism. Duplex examination releveled biphasic curves in the foot arteries. The ankle-brachial index was carried out with a result of 1.1, obtaining a WIFi stage of 3 (2/0/2). Venous disease was excluded. A biopsy was obtained, revealing small thick-walled vessels with dystrophic calcification of the intima and media. After surgical debridement, antibiotics directed at the superimposed infection were administered and local wound care with chemical debridement was performed. The ulcers healed completely after 8 weeks and there was no recurrence after 6 months. CONCLUSION: The diagnosis is made by clinical and histological findings. Treatment involves a multidisciplinary approach with medical treatment and wound care. Drugs such as sodium thiosulphate and cinacalcet have shown good results in the healing of ulcers. Vascular surgeons, as they often deal with patients with leg ulcers, should be aware of calciphylaxis as a differential diagnosis when vascular disease is excluded.
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spelling doaj.art-8ba2be6f00f24c3b9707ab9282137ec42024-03-03T23:40:55ZporSociedade Portuguesa de Angiologia e Cirurgia VascularAngiologia e Cirurgia Vascular1646-706X2183-00962024-03-0119410.48750/acv.546Calciphylaxis: a rare cause of limb ulcer – a case reportRoberto Cunha0Manuel Silva1Vítor Carneiro2Pedro Sousa3Mafalda Correia4Isabel Vieira5Isabel Cássio6Nelson Oliveira7Emanuel Dias8Serviço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, PortugalServiço de Angiologia e Cirurgia Vascular do Hospital Divino Espírito Santo, Ponta Delgada, Portugal BACKGROUND: Calciphylaxis is a rare disease with a poor prognosis predominantly found in end-stage renal disease patients treated with dialysis. This pathology most commonly presents with painful non-healing ulcers, predisposing to high risk of sepsis and death. The authors present a case of bilateral leg ulcers due to calciphylaxis. CASE REPORT: A 40-year-old female patient presented with painful ulcers in both lower limbs, for two months. She had a history of end-stage renal disease, morbid obesity, severe hypertension, arterial fibrillation and secondary hyperparathyroidism. Duplex examination releveled biphasic curves in the foot arteries. The ankle-brachial index was carried out with a result of 1.1, obtaining a WIFi stage of 3 (2/0/2). Venous disease was excluded. A biopsy was obtained, revealing small thick-walled vessels with dystrophic calcification of the intima and media. After surgical debridement, antibiotics directed at the superimposed infection were administered and local wound care with chemical debridement was performed. The ulcers healed completely after 8 weeks and there was no recurrence after 6 months. CONCLUSION: The diagnosis is made by clinical and histological findings. Treatment involves a multidisciplinary approach with medical treatment and wound care. Drugs such as sodium thiosulphate and cinacalcet have shown good results in the healing of ulcers. Vascular surgeons, as they often deal with patients with leg ulcers, should be aware of calciphylaxis as a differential diagnosis when vascular disease is excluded. https://acvjournal.com/index.php/acv/article/view/546CalciphylaxisCalcific uremic arteriolopathyEnd stage renal diseaseVascular surgery
spellingShingle Roberto Cunha
Manuel Silva
Vítor Carneiro
Pedro Sousa
Mafalda Correia
Isabel Vieira
Isabel Cássio
Nelson Oliveira
Emanuel Dias
Calciphylaxis: a rare cause of limb ulcer – a case report
Angiologia e Cirurgia Vascular
Calciphylaxis
Calcific uremic arteriolopathy
End stage renal disease
Vascular surgery
title Calciphylaxis: a rare cause of limb ulcer – a case report
title_full Calciphylaxis: a rare cause of limb ulcer – a case report
title_fullStr Calciphylaxis: a rare cause of limb ulcer – a case report
title_full_unstemmed Calciphylaxis: a rare cause of limb ulcer – a case report
title_short Calciphylaxis: a rare cause of limb ulcer – a case report
title_sort calciphylaxis a rare cause of limb ulcer a case report
topic Calciphylaxis
Calcific uremic arteriolopathy
End stage renal disease
Vascular surgery
url https://acvjournal.com/index.php/acv/article/view/546
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