Calcifilaxis / Calciphylaxis

La calcifilaxis o arteriolopatía urémica calcificante es una enfermedad rara que conlleva elevada morbilidad y una mortalidad de 40-80%. Se produce por la calcificación de los vasos de pequeño calibre y afecta sobre todo a los pacientes con insuficiencia renal crónica. Suele iniciarse con cambios en la coloración de la piel. Luego se produce ulceración, dolor y necrosis cutánea. Se presenta el caso de un varón de 70 años con insuficiencia renal crónica que desarrolló lesiones cutáneas dolorosas en los miembros inferiores.

Calciphylaxis or calcifying uremic arteriolopathy is a rare disease that carries high morbidity and mortality between 40% and 80%. It is produced by calcification of small caliber vessels and mainly affects patients with chronic renal failure. It usually begins with a change in skin color and then ulceration, pain and skin necrosis occur. We present a 70-year-old man with chronic renal failure who developed painful skin lesions on his lower limbs.

Calcifilaxis de la arteria temporal simulando arteritis temporal / Calciphylaxis of the temporal artery simulating temporal arteritis

La calcifilaxis se caracteriza por una intensa deposición de calcio en pequeños vasos sanguíneos, piel y otros órganos, descripta principalmente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción paratiroidea. Hasta la fecha, solo hay siete casos descriptos en la literatura de calcifilaxis que imita arteritis de células gigantes (ACG). En esta revisión presentamos el octavo caso documentado patológicamente.

Calciphylaxis is characterized by intense deposition of calcium in small blood vessels, skin, and other organs, described mainly in patients with chronic renal insufficiency, renal transplant of parathyroid dysfunction. To date, there are only seven cases described in literature of calciphylaxis mimicking giant cell arteritis (GCA). In this review, we present the eighth case pathologically documented.

Multiple extremity necrosis in fatal calciphylaxis: Case report / Necrose de múltiplas extremidades em quadro fatal de calcifilaxia: Relato de caso

ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

RESUMO Introdução: O impacto clínico da calcificação vascular está bem estabelecido no âmbito de morbimortalidade cardiovascular, mas outras síndromes clínicas, como a calcifilaxia, apesar de menos frequente, têm significante impacto na doença renal crônica. Métodos: Relato de caso de mulher, 27 anos, com queixa de dor em pododáctilos bilateralmente havia 3 dias, com presença de pequenas áreas necróticas nos locais referidos. Antecedente pessoal de diabetes tipo 1 (há 25 anos), com doença renal crônica, em diálise peritoneal, além de artrite reumatoide. Teve internação hospitalar, que antecedeu o quadro atual, devido à exacerbação da artrite reumatoide, evoluindo com trombo intracardíaco por complicação de cateter venoso, quando iniciou uso de varfarina. A isquemia progrediu para pés com necessidade de amputações bilaterais. Quirodáctilos também foram acometidos. Trombofilias, vasculites, endocardite ou outras fontes emboligênicas foram pesquisadas e descartadas. Anatomopatológico evidenciou: necrose de pele e partes moles superficiais com trombose arterial recente e calcificação medial de Monckeberg. Tratamento foi instituído com bisfosfonato e tiossulfato de sódio, conversão para hemodiálise e substituição de varfarina por heparina não fracionada. Apesar de toda a terapia, a paciente foi a óbito após quatro meses de evolução. Discussão: A calcifilaxia é uma rara síndrome de calcificação da microvasculatura que resulta em graves lesões isquêmicas. Tem patogênese relacionada ao distúrbio mineral e ósseo da doença renal crônica combinado com o desbalanço entre promotores e inibidores de calcificação vascular, com particular importância ao antagonismo da vitamina K. Conclusão: A estratégia preventiva é fundamental, uma vez que a terapia é complexa e de eficácia pouco validada.

Arteriolopatía urémica calcificante: presentación de caso clínico con manejo médico conservador / Calcific uremic arteriolopathy: presentation of a clinical case with conservative medical management

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)

Calcifilaxis / Calciphylaxis

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.

Calcifilaxis en terapia de sustitución renal / Calcifilaxis in renal substitution therapy

Resumen La calcifilaxis es una de las complicaciones menos comunes de la enfermedad renal crónica avanzada, sobretodo en terapia de sustitución renal, se desconoce la fisiopatología exacta de aparición, pero se cree, que es por una alteración en el metabolismo óseo-mineral. Se describe un caso clínico, de un paciente con enfermedad renal crónica, que presentó como complicación grave calcifilaxis, llegando a dicho diagnóstico gracias a las imágenes características de dicha patología tomadas del banco del servicio de imagenología del hospital. En conclusión, la calcifilaxis, a pesar de ser una patología difícil de encontrar en la actualidad, debido al mejor control del metabolismo óseo-mineral, se debe considerar en aquellos pacientes con progresión rápida de la enfermedad renal y con presencia de lesiones calcificadas supurativas en extremidades.

Abstract Calciphylaxis is one of the less common complications of Chronic Advanced Kidney Disease, especially in renal replacement therapy, the exact pathophysiology of its appearance is unknown, but it is believed that it is due to an alteration in bone-mineral metabolism. We describe a clinical case of a patient with chronic kidney disease, who presented as a serious complication calciphylaxis, reaching this diagnosis thanks to the characteristic images of this pathology taken from the bank of the Hospital's imaging service. In conclusion, calciphylaxis, despite being a pathology difficult to find nowadays due to better control of bone-mineral metabolism, should be considered especially in those patients with rapid progression of renal disease and presence of suppurative calcified lesions in extremities.

A Case Report: Total Parathyroidectomy with Autotransplantation for Secondary Hyperparathyroidism

Secondary hyperparathyroidism (HPT) usually result from parathyroid gland hyperplasia that produces excess parathyroid hormone (PTH). Decreased renal function leads to elevate serum phosphate levels and reduce vitamin D production, which results in hypocalcemia. Skeletal resistance to PTH results in persistently and frequently extremely elevated PTH levels and renal osteopathy. Treatment of choice for secondary HPT is medical management including calcitriol and vitamin D. However, for some cases in calciphylaxis and the failure including PTH >800 pg/mL or osteoporosis under maximal medical management surgical intervention could be an alternative option. We described a case of 47-year-old woman with surgical intervention for secondary hyperparathyroidism.

Sodium thiosulfate and pamidronate for treatment of calciphylaxis: case report / Tiosulfato de sodio y pamidronato para el tratamiento de calcifilaxis: reporte de caso

Abstract Introduction: Calciphylaxis is an infrequent disease that almost exclusively affects patients with chronic kidney disease, although cases have been observed in patients without renal function impairment. The diagnosis is mainly made by clinical manifestations and subsequently confirmed by radiological and histological study. The optimal treatment is not known, although there is a consensus that a multifactorial approach is required. Clinical Case: A 68-year-old woman on hemodialysis for 2 years, who presented a painful nodular lesion in the left thigh, a skin biopsy was performed resulting in a diagnosis of calciphylaxis. Treatment and Outcome: Treatment was started with intravenous sodium thiosulfate. Pamidronate is added intravenously, three months later, due to an unfavorable evolution. After 6 months of treatment, improvement in nodular lesions and healing of the ulcerated lesion was observed to be generally well tolerated treatment. Conclusion: The combined treatment of sodium thiosulfate, pamidronate and calcitomimetics has been effectiveand safe for the treatment of calciphylaxis, inducing complete remission.

Resumen: Introducción: La calcifilaxis es una enfermedad infrecuente que afecta casi exclusivamente a pacientes con insuficiencia renal, aunque se han observado casos en pacientes sin deterioro de la función renal. El diagnóstico es clínico confirmándose con estudio radiológico e histológico. No se conoce con exactitud el tratamiento óptimo, aunque hay consenso en que se requiere un abordaje multifactorial. Caso Clínico: Mujer de 68 años en hemodiálisis desde hace 2 años, que presenta una lesión nodular dolorosa en muslo izquierdo, resultando un diagnostico compatible con calcifilaxis, tras biopsia cutánea. Tratamiento y resultado: Inicia tratamiento con tiosulfato de sodio vía venosa. Tres meses más tarde y ante la evolución desfavorable, se añade al tratamiento pamidronato vía intravenosa. Tras 6 meses de tratamiento se observa mejoría de las lesiones nodulares y cicatrización de la lesión ulcerada, habiéndose experimentado buena tolerancia. Conclusión: El tratamiento combinado de tiosulfato de sodio, pamidronato y calcimiméticos ha resultado efectivo y seguro para el tratamiento de la calcifilaxis, induciendo su remisión completa.

Pigmented purpura and cutaneous vascular occlusion syndromes

Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

Calcifilaxia idiopática: relato de caso / Idiopathic calciphylaxis: case report

A calcifilaxia é uma vasculopatia obliterativa caracterizada por necrose isquêmica cutânea, com instalação aguda e progressiva, secundária à calcificação de vasos sanguíneos de pequeno ou médio calibre. Habitualmente, é uma complicação observada nos pacientes com hiperparatireoidismo secundário à insuficiência renal crônica. Sua patogênese ainda é desconhecida e, provavelmente, multifatorial. Este estudo descreve o caso de um paciente com calcifilaxia idiopática submetido ao tratamento clínico, desbridamento cirúrgico e curativos, com resolução completa após 6 meses. Apesar de ser uma afecção rara e grave na população em geral, o diagnóstico e o tratamento precoce da calcifilaxia permitem a evolução clínica favorável e a melhoria da qualidade de vida do paciente.(AU)

The calciphylaxis is an obliterative vasculopathy characterized by cutaneous ischemic necrosis, with acute and progressive installation, secondary to calcification of small or medium-sized blood vessels. Usually, is a complication observed in patients with hyperparathyroidism secondary to chronic renal failure. Its pathogenesis is still unknown and probably multifactorial. This study describes a case of a patient with idiopathic calciphylaxis, which was submitted to clinical treatment, surgical debridement and dressings, with complete resolution after 6 months. Although being a rare and serious disease in the general population, the early diagnosis and treatment of calciphylaxis allow favorable clinical evolution and improvement of patient's quality of life.(AU)

¿Qué hay de nuevo en calcifilaxia? / ¿What is new in calciphylaxis? / ¿O que há de novo na calcifilaxia?

Objetivo: Revisar la literatura científica publicada en las principales bases de datos, entre los años 1962 y 2016, cuyo objetivo fue abordar el tema de la calcifilaxia y sus principales características. Metodología: Se llevó a cabo una revisión de la literatura en las principales bases de datos de las Ciencias de la Salud y las Ciencias Sociales (medline-pubmed, cinhal, web of science, lilacs, sociological abstracts, cuiden, embase, psycoinfo e isi web of knowledge). Se emplearon descriptores en español, inglés y portugués: Calcifilaxis, diálisis, hiperparatiroidismo, necrosis, gangrena, calcificación vascular, cuidados de enfermería y diagnósticos de enfermería. Se hizo una revisión sistemática de los artículos seleccionados utilizando un protocolo para extraer los datos. Resultado: Se encontraron 45 artículos que cumplían con los criterios de búsqueda establecidos, 35 reportaron los resultados de ensayos clínicos o estudios cualitativos, 4 correspondían a revisiones sistemáticas y 6 se catalogaron como estudios retrospectivos. Los autores señalaron las principales características de la enfermedad, de los pacientes y los tratamientos más usados, pero poco del cuidado que debe brindar el profesional enfermero. Conclusión: En los estudios analizados se identificaron principalmente aspectos sobre el curso de la enfermedad y el tratamiento; se reportó la efectividad de algunos tratamientos utilizadas en la atención de dichos pacientes, pero ninguna de ellas definitiva, lo que permite proponer algunas acciones desde la disciplina enfermera.

Objective: To analyze the scientifi c literature published in the main databases between 1962 and 2016, whose objective was to address the issue of calciphylaxis and its main characteristics? Methodology: A literature review was carried out in the main databases of the Health Sciences and Social Sciences (medline-pubmed, cinhal, web of science, lilacs, sociological abstracts, cuiden, embase, psycoinfo and isi web of knowledge). An analytical review of the selected articles was made using a protocol to extract the data. Results: 45 articles were found that fulfi lled the established search criteria, 35 reported the results of clinical trials or qualitative studies, 4 corresponded to systematic reviews and 6 were classifi ed as retrospective studies. The authors pointed out the main characteristics of the disease, the patients and the most used treatments, but little of the care that should be provided by the nurse practitioner. Conclusion: In the analyzed studies, aspects regarding the course of the disease and treatment were identifi ed; it was reported the effectiveness of some treatments used in the care of these patients, but none of them defi nitive, which allows to propose some actions from the nurse discipline.

Objetivo: Revisar a literatura científica publicada nas principais bases de dados, entre os anos 1962 e 2016, cujo objetivo era abordar o tema da calcifilaxia e suas principais características. Metodologia: Uma revisão da literatura foi realizada nas principais bases de dados das Ciências da Saúde e das Ciências Sociais (Medline-Pubmed, Cinhal, Web of Science, Lilacs, Sociological abstracts, Cuiden, Embase, Psycoinfo e Isi Web of Knowledge). Foram usados descritores em espanhol, inglês e português: calcifilaxia, diálise, hiperparatiroidismo, necrose, gangrena, calcificação vascular, cuidados de enfermagem e diagnósticos de enfermagem. Foi feita uma revisão sistemática dos artigos selecionados utilizando um protocolo para extrair os dados. Resultados: Encontraram-se 45 artigos que cumpriam com os critérios de busca estabelecidos, 35 reportaram os resultados de ensaios clínicos ou estudos qualitativos, quatro (4) correspondiam a revisões sistemáticas e seis (6) se catalogaram como estudos retrospectivos. Os autores apontaram as principais características da doença, dos pacientes e os tratamentos mais usados, mas pouco do cuidado que debe oferecer o professional da enfermagem. Conclusão: Nos estudos analisados se identificaram principalmente aspectos sobre o curso da doença e o tratamento; se reportou a efetividade de alguns tratamentos utilizados no atendimento desses pacientes, mas nenhum deles definitivo, o que permite propor algumas ações desde a profissão da enfermagem.

Early diagnosis, prevention and treatment for calcific uremic arteriolopathy / 中南大学学报(医学版)

Calcific uremic arteriopathy (CUA), termed calciphylaxis, is a rare but highly fatal clinical syndrome. There is no clearly laboratory diagnostic criteria for CUA. The medium and small arterial calcification and microthrombosis discovered by skin biopsy, radiologic imaging，bone scan and the evidence of activation of the bone morphogenetic protein signal (BMPs) transduction pathway are useful for early diagnosis of this disease. The common therapies (including intravenous sodium thiosulfate (STS) and bisphosphonates, hyperbaric oxygen therapy and other symptomatic supports) are used for the management of wounds, pain, nutrition, dialysis and so on. Controlling the chronic kidney disease-mineral and bone disorder (CKD-MBD) and some complications of dialysis and drugs (such as warfarin, active vitamin D) can prevent CUA. However, CUA patients still have poor prognosis and high mortality. Since some patients progress rapidly, it is of great importance to make early diagnosis and provide effective treatments with multidisciplinary management.

Histopathologic review of calcemic uremic arteriolopathy: A case series

@#<p style="text-align: justify;">Calcemic uremic arteriolopathy or calciphylaxis is an uncommon disorder presenting clinically as skin ischemia and necrosis, and histologically as vascular calcification and thrombosis of dermal and subdermal vasculature. This study described two Filipino females with end-stage renal disease on chronic dialysis with non-healing ulcers on the lower extremities as a result of calcification in the vessels of the dermis and subcutaneous fat with associated fat necrosis. Current understanding of its various histologic features was reviewed for proper diagnosis.</p>

Calcifilaxis / Calciphylaxis

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.

Calcific uraemic arteriolopathy (calciphylaxis) in patients on renal replacement therapy

Background. Calcific uraemic arteriolopathy (calciphylaxis) is an unusual and potentially fatal condition characterised by small-vessel calcification and ischaemic skin necrosis. It mainly affects patients with end-stage renal disease (ESRD) on haemodialysis, but may rarely occur in the absence of ESRD in conditions such as primary hyperparathyroidism, malignancy, alcoholic liver disease and connective tissue disease.Methods. We reviewed the records of all patients diagnosed with calciphylaxis while on renal replacement therapy at Tygerberg Hospital, Cape Town, South Africa, between 1990 and 2014, to describe its presentation, course and final outcome.Results. Nineteen patients developed calciphylaxis over this period. Their median age was 34 years and 13 (68.4%) were female. Fifteen (78.9%) had received a kidney transplant. All patients had painful skin lesions that rapidly progressed to infarction. Small-vessel calcification was seen on skin biopsy in 13 patients. Twelve patients had hyperparathyroidism. Several of the transplanted patients had been treated for graft rejection in the year preceding the diagnosis. Treatment consisted of good wound care and efforts to normalise serum calcium and phosphate levels. Five patients received an urgent parathyroidectomy. The outcome was fatal in 17 patients, with sepsis being the main cause of death.Conclusions. In our patients, calciphylaxis carried a worse prognosis than previously reported internationally. It should always be considered in the differential diagnosis of painful skin lesions in the dialysis or transplant patient

Idiopathic Calcinosis Cutis of the Scrotum: A Case Report

Calcinosis cutis-calcification in soft tissue-is a rare benign disease that is separated into the following subtypes: dystrophic, iatrogenic, metastatic, calciphylaxis, and idiopathic. One of common site of calcinosis cutis is the scrotum. The nodules slowly grow for years or decades. The characteristic of calcinosis cutis of the scrotum is generally asymptomatic, yellowish marble-like, hard, polypoidal, solitary, or multiple. However, the pathogenesis of this nodule remains ambiguous and controversial. Thus, we reviewed possible causes and therapeutic consideration of calcinosis cutis of the scrotum.

Calcifilaxis: revisión de tema acerca de la calcifilaxis del pene / Calciphylaxis: review of the topic about penile calcifilaxis

Resumen:La calcifilaxis es una alteración vascular considerada como un síndrome multifactorial, que afecta la túnica media de las arterias más pequeñas causando isquemia del pene y esto a su vez una gangrena de rápida evolución.Su etiopatogenia aun no es muy clara y la bibliografía acerca de esta condición es escasa, a pesar de que su incidencia ha ido en aumento en los últimos años. La calcifilaxis del pene es una condición casi exclusiva para pacientes con insuficiencia renal crónica que reciben diálisis y frecuentemente es subdiagnosticada por los profesionales de la salud. Sus manifestaciones clínicas son altamente agresivas y de rápida evolución, además asocia una alta morbi-mortalidad por lo que debe diagnosticarse con la mayor brevedad posible. El manejo de los pacientes con calcifilaxis es multidisciplinario, en el intervienen tanto el urólogo como el nefrólogo y su tratamiento debe personalizarse según las condiciones de cada paciente, iniciando como primera medida con un manejoconservador y posteriormente quirúrgico si no se logra la respuesta deseada.

Abstract:Calciphylaxis is a vascular disorder, considered a multifactorial syndrome that affects the media of small caliber vessels, causing penile ischemia and in turn, rapidly evolving gangrene. Despite the raising incidence rates in recent years, literary information is limited, and its pathogenesis remains unclear. This condition is almost entirely attributed to chronic kidney disease patients receiving dialysis treatment, and is often clinically underdiagnosed by health care professionals. Clinically, calciphylaxis is considered to be rapidly evolving and highly aggressive, associated with high morbility and mortality rates, making prompt diagnosis imperative.