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1.
Am J Case Rep ; 22: e930026, 2021 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-34097671

RESUMO

BACKGROUND Calciphylaxis is a rare and lifethreatening syndrome characterized by small vascular calcifications, which lead to the occlusion of blood vessels and painful skin lesions with tissue necrosis. Although the disease can develop in a population without kidney failure, it is typically detected in patients receiving dialysis, with an increasing frequency ranging from 1% to 4%. Therefore, the disease is also known as calcific uremic arteriolopathy. The prognosis in patients with coexisting chronic kidney disease is very poor, with a 1-year mortality rate of up to 80%. Numerous risk factors for calciphylaxis have been described, such as obesity, diabetes mellitus, female sex, White race, overuse of calcium and vitamin D supplements, and vitamin K deficiency. The disease is often accompanied by disorders such as hyperphosphatemia, elevated parathyroid hormone level, and a deficiency of natural calcification inhibitors, such as fetuin-A and matrix Gla protein. However, not all patients with calciphylaxis have the abnormalities described above, suggesting that the pathogenesis of calciphylaxis is multifactorial and unfortunately still uncertain. CASE REPORT We report a case of calciphylaxis in a 52-year-old White woman with multiple comorbidities and on chronic hemodialysis treatment, who presented with severe subcutaneous painful nodules and necrotic ulcers on both legs. CONCLUSIONS Although the prognosis of this rare and underrecognized disease is poor, an early diagnosis and interdisciplinary treatment including pain relief, wound care, appropriate nutritional support, correction of mineral parameters, administration of sodium thiosulphate, and adequate hemodialysis therapy can improve patient quality of life.


Assuntos
Calciofilaxia , Falência Renal Crônica , Calcificação Vascular , Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Feminino , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Pessoa de Meia-Idade , Qualidade de Vida , Diálise Renal , Calcificação Vascular/complicações
2.
BMJ Case Rep ; 14(5)2021 May 11.
Artigo em Inglês | MEDLINE | ID: mdl-33975849

RESUMO

Calcinosis cutis is an unusual disorder characterised by calcium-phosphate deposition into cutaneous and subcutaneous tissues. There are five subtypes: dystrophic, metastatic, idiopathic, iatrogenic and calciphylaxis. Our objective was to report a patient with a history of haemodialysis-dependent end-stage renal disease (ESRD) who developed metastatic calcinosis cutis, which is usually associated with abnormalities of calcium-phosphate metabolism in renal failure. In our patient, the serum calcium and phosphate levels were normal. Additionally, we describe the diagnostic approach and the difficulties involved in the management of calcinosis cutis in patients with haemodialysis-dependent ESRD.


Assuntos
Calcinose , Calciofilaxia , Transplante de Rim , Dermatopatias , Calcinose/etiologia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Humanos , Pele , Dermatopatias/etiologia
3.
BMJ Case Rep ; 14(4)2021 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-33827879

RESUMO

Calciphylaxis is commonly associated with end-stage renal disease (ESRD) and renal transplant. We present a rare case of early onset calciphylaxis in a patient presenting with acute kidney injury (AKI) secondary to anti-glomerular basement membrane (anti-GBM) antibody disease. A 65-year-old obese Caucasian woman with type 2 diabetes mellitus and hypertension presented with a 1-month history of painless gross haematuria and worsening lower extremity oedema. Laboratory results indicated AKI and nephrotic-range proteinuria. Anti-glomerular antibodies were elevated. Renal biopsy revealed focal crescentic glomerulonephritis with linear capillary immunoglobulin G staining consistent with anti-GBM antibody disease. She was treated with haemodialysis, plasmapheresis, steroids, bumetanide and cyclophosphamide. Two months later, she developed necrotic lesions on bilateral thighs. Wound biopsy was consistent with calciphylaxis. This case highlights that calciphylaxis, usually seen in patients with chronic kidney disease or ESRD, can manifest in patients with AKI as well.


Assuntos
Injúria Renal Aguda , Doença Antimembrana Basal Glomerular , Calciofilaxia , Diabetes Mellitus Tipo 2 , Glomerulonefrite , Injúria Renal Aguda/etiologia , Idoso , Doença Antimembrana Basal Glomerular/complicações , Autoanticorpos , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Feminino , Hemorragia , Humanos , Pneumopatias
5.
Rev Med Suisse ; 17(727): 394-398, 2021 Feb 24.
Artigo em Francês | MEDLINE | ID: mdl-33625805

RESUMO

Calciphylaxis is a rare but devastating condition characterized by a calcifying thrombosing microangiopathy resulting in painful necrotic skin lesions. Risk factors are multiple, the most important being obesity, disorders of phosphocalcic metabolism and acenocoumarol. Largely unknown by the medical community, its pathogenesis is still incompletely elucidated; its diagnosis by skin biopsy remains difficult and increasingly debated because potentially associated with an aggravation of lesions. Its treatment must be as premature as possible andmultimodal. However, the results are up to now unsatisfactory, as specific treatment of calciphylaxis does not yet exist.


Assuntos
Calciofilaxia , Doenças Vasculares , Biópsia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Feminino , Humanos , Rim , Necrose , Gravidez
6.
Dermatol Ther ; 34(2): e14769, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33421241

RESUMO

Calciphylaxis is a rare condition characterized by skin ulceration and necrosis as a result of vascular calcification of the small and medium blood vessels of skin and subcutaneous tissues. It mainly occurs in patients with advanced chronic kidney disease and sometimes leads to complications with a fatal outcome. In this report, we describe the case of a 67-year-old male patient with end stage renal disease presenting painful skin ulcers on his lower limbs. The lesions had progressively grown and were associated to severe pain and decreased quality of life. The ulcers did not respond to conventional treatments and the patient underwent skin biopsy of these lesions obtaining anatomopathological findings compatible with calciphylaxis. In this report, we present an innovative treatment for skin ulcers secondary to calciphylaxis using cryopreserved amniotic membrane (AM) as a dressing in order to promote epithelialization of the wounds. After four applications, healing of the main ulcer and reduction in pain was achieved. In summary, applying cryopreserved AM probed to be a promising strategy to reduce pain and to enhance epithelialization and healing of chronic non-responsive ulcers in calciphylaxis.


Assuntos
Calciofilaxia , Falência Renal Crônica , Úlcera Cutânea , Idoso , Âmnio , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Masculino , Qualidade de Vida , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/etiologia , Úlcera Cutânea/terapia
7.
Rev. méd. hondur ; 88(2): 115-119, jul.-dic. 2020. tab., ilus
Artigo em Espanhol | LILACS | ID: biblio-1179039

RESUMO

Antecedentes: La arteriolopatia calcificante urémica o calcifilaxis es un síndrome raro, potencialmente mortal, que afec-ta casi en exclusiva a pacientes con insuficiencia renal y diálisis, caracterizado por calcificación vascular de arterias de pequeño y mediano calibre, con posterior proliferación, fibrosis y trombosis que conducen finalmente a necrosis y úlceras cutáneas. Se asocia con la enfermedad renal crónica terminal y trasplante renal, con preva-lencia de 1-4% de los pacientes con insuficiencia renal crónica. El tratamiento es especializado a base de cámara hiperbárica y para-tiroidectomía para inducir curación. Descripción del caso clínico: Femenina de 42 años, captada en la consulta externa de nefrología en el Instituto Hondureño de Seguridad Social en el año 2017, con antecedente de hipertensión arterial y nefropatía crónica, sometida a trasplante renal en 1998 el cual fue fallido, posteriormente en pro-grama de hemodiálisis y manejo conservador desde el año 2005. La paciente desarrolló lesiones equimóticas en tronco y úlceras en sitios de nódulos subcutáneos que se sobreinfectaron, desarrollan-do signos de respuesta inflamatoria sistémica. Los exámenes de laboratorio mostraron hiperfosfatemia, paratohormona 3518 pg/ml, producto calcio-fósforo 73.5. Ante la falta de manejo quirúrgico (pa-ratiroidectomía) y cámara hiperbárica en la institución, en el 2017 se estableció tratamiento conservador a base de antibióticos, analgési-cos, y hemodiálisis diarias, con lo que presentó mejoría del cuadro clínico; sin embargo, sin resolución de su cuadro de base de la calci-filaxis. Conclusión: El manejo conservador en el caso de pacientes con calcifilaxis es una opción de tratamiento disponible con buena respuesta en pacientes con seguimiento estrecho...(AU)


Assuntos
Humanos , Feminino , Adulto , Calciofilaxia/diagnóstico , Calcificação Vascular , Insuficiência Renal , Hiperparatireoidismo Secundário
10.
Rev. argent. dermatol ; 101(1): 61-70, mar. 2020. graf
Artigo em Espanhol | LILACS | ID: biblio-1125807

RESUMO

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.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Sepse/prevenção & controle , Calciofilaxia/mortalidade , Diagnóstico Diferencial , Necrose/complicações
11.
Cutis ; 105(1): E11-E14, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32074157

RESUMO

Calciphylaxis is a potentially fatal disease caused by metastatic calcification of the small- and medium-sized blood vessels of the dermis and subcutis. It most commonly is seen in patients with renal disease requiring dialysis, but it also may be triggered by nonuremic causes in patients with known risk factors for calciphylaxis. We report a case of nonuremic calciphylaxis (NUC) occurring in the setting of multiple risk factors, including chronic corticosteroid use, obesity, rapid weight loss, and hypotension. A review of the literature also is provided with an in-depth discussion of the known risk factors and triggers of NUC.


Assuntos
Calciofilaxia/diagnóstico , Hipotensão/complicações , Perda de Peso/fisiologia , Idoso , Calciofilaxia/etiologia , Feminino , Glucocorticoides/administração & dosagem , Humanos , Obesidade/complicações , Fatores de Risco
12.
Ann R Coll Surg Engl ; 102(2): e36-e38, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31530167

RESUMO

Penile calciphylaxis or calcific uremic arteriolopathy is a rare urological condition often associated with patients undergoing renal dialysis for end-stage renal disease. The majority of cases are associated with systemic calciphylaxis. The pathophysiology, diagnosis and management of penile calciphylaxis as an individual entity has brought little attention. The rates of comorbidity and mortality of these patients are often particularly high. Early diagnosis and a multidisciplinary approach are therefore essential. We report a case of penile calciphylaxis in a 59-year-old man with end-stage renal failure on haemodialysis who was successfully managed conservatively.


Assuntos
Angioplastia/métodos , Calciofilaxia/terapia , Tratamento Conservador/métodos , Doenças do Pênis/terapia , Inibidores da Agregação Plaquetária/uso terapêutico , Bandagens , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Artéria Femoral/cirurgia , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Doenças do Pênis/diagnóstico , Doenças do Pênis/etiologia , Pênis/irrigação sanguínea , Pênis/patologia , Diálise Renal/efeitos adversos , Resultado do Tratamento
13.
J Dermatolog Treat ; 31(5): 545-548, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31075991

RESUMO

Background: Calciphylaxis is associated with end-stage renal failure or kidney transplant, although, cases of non-uremic calciphylaxis have been reported. It is a rare disorder, with high mortality, characterized by vascular calcification within cutaneous vessels; vascular calcification can occur in other organs, besides skin, like heart, lung, or pancreas, which is named visceral calciphylaxis.Objective: The purpose was to review current knowledge regarding diagnosis and therapeutic approach to cutaneous calciphylaxis.Methods: A literature review has been conducted associated to word "calciphylaxis".Results: The diagnosis is based mainly on clinical features and histologic findings. Therapeutic options are still controversial.Conclusions: Cutaneous calciphylaxis is a puzzling disease, with a challenging diagnosis, and a complex treatment, which requires a multidisciplinary team and expertise.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Cálcio/metabolismo , Guias como Assunto , Humanos , Oxigenação Hiperbárica , Falência Renal Crônica/complicações , Falência Renal Crônica/patologia , Transplante de Rim/efeitos adversos , Pele/patologia
14.
Adv Chronic Kidney Dis ; 26(6): 484-490, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31831126

RESUMO

Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. Although mostly described in dialysis patients, it can affect patients with normal kidney function. The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. Late diagnosis and advanced lesions can significantly shorten life expectancy. Calciphylaxis wounds can have a major influence on the quality of life of patients, usually due to the immense unbearable pain these patients suffer from. The management of calciphylaxis mainly comprises aggressive wound care and symptomatic management. Therapeutic options are few and far between and are limited to off-label uses. Recent understanding of the pathogenesis of lesions has enabled development of novel therapeutic options, some of which are being studied in clinical trials (sodium thiosulfate, vitamin K). Vascular calcification and thrombosis underlie development of these lesions and research has been aimed at studying drugs that counteract such processes. Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Calcificação Vascular/tratamento farmacológico , Calciofilaxia/complicações , Quelantes/uso terapêutico , Humanos , Falência Renal Crônica/terapia , Microvasos , Manejo da Dor , Diálise Renal , Tiossulfatos/uso terapêutico , Trombofilia/tratamento farmacológico , Calcificação Vascular/etiologia , Calcificação Vascular/metabolismo , Ferimentos e Lesões/etiologia , Ferimentos e Lesões/terapia
15.
BMJ Case Rep ; 12(12)2019 Dec 05.
Artigo em Inglês | MEDLINE | ID: mdl-31811092

RESUMO

Penile calciphylaxis is a rare but clinically challenging condition to manage. While multiple treatment options have been proposed, the underlying evidence is anecdotal, and the overall prognosis remains extremely poor. We describe the case of a patient who underwent urgent debridement of a suspected gangrenous penile tip, who was intraoperatively found to have penile calciphylaxis. The necrosis progressed and the patient had a glans amputation. A non-healing wound developed, requiring reconstruction with two skin grafts using foreskin by the plastic and reconstructive surgical unit. The patient was commenced on sodium thiosulfate and his wound had completely healed 2 months following his last operation. We review the literature to determine the biochemical features, management options and outcomes in patients with penile calciphylaxis.


Assuntos
Calciofilaxia/diagnóstico , Diabetes Mellitus Tipo 2 , Falência Renal Crônica , Doenças do Pênis/diagnóstico , Pênis/patologia , Adulto , Amputação , Calciofilaxia/complicações , Calciofilaxia/cirurgia , Diagnóstico Diferencial , Gangrena , Humanos , Masculino , Doenças do Pênis/complicações , Doenças do Pênis/cirurgia
16.
Am J Surg Pathol ; 43(9): 1273-1281, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31192861

RESUMO

Diagnosis of calciphylaxis is crucial, yet its distinction from other vascular diseases can be challenging. Although vascular calcification and thrombosis are hallmarks of calciphylaxis, the incidence and patterns of these features in other vascular diseases have not been well characterized. The specificity of fine calcium deposits in vessel walls (identifiable on von Kossa [VK] stain only) and other extravascular calcifications is not entirely clear. We retrospectively examined the clinicopathologic features in calciphylaxis (n=27), gangrene and viable skin at amputation margin (n=20 each), chronic stasis (n=22), and thrombotic vasculopathy (n=19) to identify useful discriminators. Calcification of subcutaneous small vessels appreciable on hematoxylin and eosin stain was relatively specific for calciphylaxis, although sensitivity was low (56%). VK detected fine calcium deposits in vessel walls not appreciable on hematoxylin and eosin, however, specificity was limited by frequent finding of similar deposits in peripheral artery disease. Combining calcium deposits detected by VK and thrombosis of subcutaneous small vessels resulted in optimal sensitivity (85%) and specificity (88%) for calciphylaxis. Similar observations applied to medium-sized vessel calcification. Calcification of eccrine gland basement membranes, elastic fibers, and perineurium did not effectively distinguish calciphylaxis from other groups. Diffuse dermal angiomatosis was exclusively found in calciphylaxis in this study. In conclusion, VK is useful in enhancing the detection of vascular calcification and avoiding the false-negative diagnosis, but this finding requires concomitant subcutaneous small vessel thrombosis to support a diagnosis of calciphylaxis. Diffuse dermal angiomatosis should increase suspicion for underlying calciphylaxis and prompt deeper sampling in the appropriate clinical setting.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/patologia , Adulto , Idoso , Feminino , Gangrena/diagnóstico , Gangrena/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/diagnóstico , Doença Arterial Periférica/patologia , Coloração e Rotulagem , Trombose/diagnóstico , Trombose/patologia , Doenças Vasculares/diagnóstico , Doenças Vasculares/patologia
17.
J Wound Care ; 28(Sup6): S1-S92, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-31169055

Assuntos
Ferimentos e Lesões/terapia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Transtornos da Coagulação Sanguínea/complicações , Transtornos da Coagulação Sanguínea/diagnóstico , Transtornos da Coagulação Sanguínea/patologia , Transtornos da Coagulação Sanguínea/terapia , Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Calciofilaxia/patologia , Calciofilaxia/terapia , Eritema Endurado/complicações , Eritema Endurado/diagnóstico , Eritema Endurado/patologia , Eritema Endurado/terapia , Transtornos Autoinduzidos/complicações , Transtornos Autoinduzidos/diagnóstico , Transtornos Autoinduzidos/terapia , Hidradenite Supurativa/complicações , Hidradenite Supurativa/diagnóstico , Hidradenite Supurativa/patologia , Hidradenite Supurativa/terapia , Humanos , Livedo Reticular/complicações , Livedo Reticular/diagnóstico , Livedo Reticular/patologia , Livedo Reticular/terapia , Equipe de Assistência ao Paciente , Poliarterite Nodosa/complicações , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/patologia , Poliarterite Nodosa/terapia , Púrpura de Schoenlein-Henoch/complicações , Púrpura de Schoenlein-Henoch/diagnóstico , Púrpura de Schoenlein-Henoch/patologia , Púrpura de Schoenlein-Henoch/terapia , Pioderma Gangrenoso/complicações , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/patologia , Pioderma Gangrenoso/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/secundário , Neoplasias Cutâneas/terapia , Vasculite/complicações , Vasculite/diagnóstico , Vasculite/patologia , Vasculite/terapia , Ferimentos e Lesões/diagnóstico
18.
Curr Opin Nephrol Hypertens ; 28(5): 448-454, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31169527

RESUMO

PURPOSE OF REVIEW: Calciphylaxis is a disorder of cutaneous microvascular calcification and thrombosis leading to chronic, excruciatingly painful, progressive wounds with a high risk of sepsis and death. The diagnosis and treatment of calciphylaxis presents significant challenges. A poorly understood disease, the management of calciphylaxis has mostly been restricted to wound management and a few novel therapies. Data from patient registries and new studies on causal pathways is stimulating the development of pathogenesis-based medical therapies. RECENT FINDINGS: Much needed clinical trials are now underway to examine the safety and efficacy of sodium thiosulfate and other therapeutics for the indication of calciphylaxis. There is emerging data suggesting a potential role of therapeutic anticoagulation in these patients. There has also been a renewed emphasis on patient-oriented outcomes, such as improvement of pain scores and quality-of-life indices. SUMMARY: This review highlights ongoing clinical trials studying therapeutic options in calciphylaxis and emphasizes the causal pathways that led to the development of such therapies.


Assuntos
Calciofilaxia/terapia , Anticoagulantes/uso terapêutico , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Cinacalcete/uso terapêutico , Ensaios Clínicos como Assunto , Humanos , Manejo da Dor , Paratireoidectomia , Tiossulfatos/uso terapêutico
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