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1.
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
3.
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
4.
G Ital Nefrol ; 37(1)2020 Feb 12.
Artigo em Italiano | MEDLINE | ID: mdl-32068359

RESUMO

Primary hyperoxaluria (PH) is a rare genetic disorder with autosomal recessive transmission, characterized by high endogenous production and markedly excessive urinary excretion of oxalate (Ox). It causes the accumulation of calcium oxide crystals in organs and tissues including bones, heart, arteries, skin and kidneys, where it may cause oxalo-calcic nephrolithiasis, nephrocalcinosis and chronic renal failure. Some forms are secondary to enteric diseases, drugs or dietetic substances, while three primitive forms, caused by various enzymatic defects, are currently known: PH1, PH2 and PH3. An early diagnosis, with the aid of biochemical and genetic investigations, helps prevent complications and establish a therapeutic strategy that often includes liver and liver-kidney transplantation, improving the prognosis of these patients. In this work we describe the clinical case of a patient with PH1 undergoing extracorporeal hemodialysis treatment and we report the latest research results that could change the life of patients with PH.


Assuntos
Calciofilaxia/terapia , Hiperoxalúria Primária/genética , Hiperoxalúria Primária/terapia , Diálise Renal/métodos , Dermatopatias Metabólicas/terapia , Transaminases/genética , Calciofilaxia/etiologia , Calciofilaxia/patologia , Compostos de Cálcio/metabolismo , Feminino , Glioxilatos/metabolismo , Hemodiafiltração/métodos , Humanos , Hiperoxalúria Primária/diagnóstico , Falência Renal Crônica/etiologia , Transplante de Rim , Pessoa de Meia-Idade , Nefrocalcinose/etiologia , Nefrocalcinose/terapia , Uso Off-Label , Oxalatos/metabolismo , Óxidos/metabolismo , Dermatopatias Metabólicas/etiologia , Dermatopatias Metabólicas/patologia , Tiossulfatos/uso terapêutico
5.
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
6.
J Am Acad Dermatol ; 82(4): 799-816, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31479690

RESUMO

In this article we focus on updates in select etiologies of retiform purpura. These causes of retiform purpura, in addition to bacterial or fungal sepsis, disseminated intravascular coagulation, purpura fulminans, and catastrophic antiphospholipid syndrome, are important diagnoses with potential for morbidity and mortality. Important aspects in the pathophysiology, patient demographics and risk factors, updates in the diagnostic workup, histopathology, and treatment of these specific conditions are discussed.


Assuntos
Púrpura/diagnóstico , Púrpura/etiologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/etiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Calciofilaxia/complicações , Calciofilaxia/patologia , Calciofilaxia/fisiopatologia , Calciofilaxia/terapia , Crioglobulinemia/complicações , Crioglobulinemia/patologia , Crioglobulinemia/fisiopatologia , Crioglobulinemia/terapia , Humanos , Púrpura/fisiopatologia , Púrpura/terapia , Fatores de Risco , Dermatopatias Vasculares/fisiopatologia , Dermatopatias Vasculares/terapia , Vasculite Sistêmica/complicações , Vasculite Sistêmica/patologia , Vasculite Sistêmica/fisiopatologia , Vasculite Sistêmica/terapia
7.
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
8.
Wounds ; 32(11): 294-298, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33465045

RESUMO

INTRODUCTION: Calciphylaxis is a rare, highly morbid pathological syndrome of vascular calcification and tissue necrosis. It is predominantly seen in patients with end-stage renal disease (ESRD) on chronic dialysis. There is no definitive standard of care for calciphylaxis, and the overall prognosis for patients, particularly those with ulcerated lesions, is bleak. One important role of wound care clinicians during the COVID-19 pandemic is to ensure that the continuity of care of an at-risk population is maintained while limiting the patient's potential exposure to the virus. Innovative therapies paired with alternative treatment sites of service are one such method. CASE REPORT: A 56-year-old female with ESRD on at-home peritoneal dialysis (PD) presented to the outpatient wound clinic with a punch biopsy-proven calciphylaxis lesion. Within days, state-wide "shelter-at-home" orders due to the COVID-19 pandemic went into effect. To prevent disruption in care and to minimize risk to the patient, the lesion was treated with bi-weekly self-application of a continuous topical oxygen therapy (cTOT) device paired with weekly telemedicine visits. The wound completely resolved after 9 weeks of topical oxygen therapy with no complications or device malfunctions. CONCLUSIONS: This case, to the authors' knowledge, is the first to document healing in a calciphylaxis wound with the use of cTOT. Topical oxygen therapy may be a beneficial adjunctive therapy in the treatment of wounds caused by calciphylaxis. Finding creative ways to navigate this current health care crisis is essential to help mitigate risk for vulnerable patients with advanced comorbidities.


Assuntos
COVID-19/complicações , Calciofilaxia/terapia , Oxigênio/uso terapêutico , Diálise Peritoneal/efeitos adversos , Ferimentos e Lesões/terapia , Administração Tópica , Calciofilaxia/complicações , Calciofilaxia/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Oxigênio/administração & dosagem , Quarentena , Cicatrização , Ferimentos e Lesões/etiologia
9.
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
10.
G Ital Nefrol ; 36(5)2019 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-31580546

RESUMO

Calcific uremic arteriolopathy (CUA) is a highly morbid condition usually found in ESRD patients that has rarely been reported after renal transplantation and renal function restoration. Furthermore, little is known about the optimal management of CUA in this setting. Herein, we report on the clinical case of AB, a 70-year-old woman who developed CUA after renal transplantation and renal function restoration. However, other risk factors for CUA such as diabetes and warfarin treatment, due to mechanical aortic valve implantation, were present. Thirty-eight months after renal transplantation she developed erythema and livedo reticularis in both legs and a gradually enlarging skin ulcer in the right leg. A skin biopsy of the ulcer showed features compatible with the CUA, such as sub-intimal calcification and luminal obstruction of the small dermal arterioles, tissue ischemia and signs of adipocytes degeneration. A multidisciplinary approach was adopted, including medical and non-medical treatments such as surgical debridement and vacuum-assisted closure therapy. Medical treatments included a five weeks course of once a week intravenous infusion of pamidronate and intravenous sodium thiosulfate (STS) at increasing doses. Four months after beginning the therapy with STS, a complete healing of the ulcer on the right leg and the disappearance of the livedo reticularis on the left leg was noted. In conclusion, although rare CUA may develop also in renal transplanted patients, a timely and combined therapeutic approach is essential for its resolutive treatment. Sodium thiosulfate therapy has proven to be effective and tolerated.


Assuntos
Calciofilaxia/terapia , Transplante de Rim/efeitos adversos , Úlcera da Perna/terapia , Doenças Raras/terapia , Idoso , Anticoagulantes/uso terapêutico , Conservadores da Densidade Óssea/administração & dosagem , Calciofilaxia/etiologia , Quelantes/administração & dosagem , Terapia Combinada/métodos , Diabetes Mellitus , Feminino , Humanos , Úlcera da Perna/etiologia , Livedo Reticular/etiologia , Livedo Reticular/terapia , Pamidronato/administração & dosagem , Doenças Raras/etiologia , Fatores de Risco , Tiossulfatos/administração & dosagem , Varfarina/uso terapêutico
11.
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
12.
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
14.
Adv Skin Wound Care ; 32(5): 205-215, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31008757

RESUMO

GENERAL PURPOSE: To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant should be better able to:1. Recognize the pathogenesis and clinical features of and risk factors for calciphylaxis.2. Explain the diagnosis and management of a patient with calciphylaxis. ABSTRACT: Calciphylaxis is a cutaneous ischemic infarct caused by total occlusion of blood vessels initiated by vascular calcification. Until recently, treatments have been limited to controlling its risk factors and optimizing wound care. However, recent advances in clinical understanding of the mechanism of calciphylaxis have identified promising potential therapeutic targets. This article is a narrative review summarizing the clinical features, diagnosis, pathogenesis, and treatment of calciphylaxis.


Assuntos
Calciofilaxia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/patologia , Calciofilaxia/terapia , Quelantes/uso terapêutico , Humanos , Falência Renal Crônica/complicações , Transplante de Rim/efeitos adversos , Manejo da Dor , Diálise Renal , Fatores de Risco , Tiossulfatos/uso terapêutico , Trombose , Calcificação Vascular/prevenção & controle , Calcificação Vascular/terapia , Vitamina K/uso terapêutico , Deficiência de Vitamina K/complicações , Cicatrização
15.
Saudi J Kidney Dis Transpl ; 30(1): 215-220, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30804284

RESUMO

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare, life-threatening complication of end-stage renal disease. However, it may also occur in patients without renal failure, the nonuremic calciphylaxis. Most patients present with painful skin ulcers. Delayed diagnosis can lead to sepsis-related morbidity and mortality. Aberrations in calcium, phosphate, and parathyroid hormone physiology are common and biopsy is diagnostic. Early diagnosis and correction of vascular and metabolic aberrations may assist in healing as may the use of sodium thiosulfate and hyperbaric oxygen therapy. We report on successful treatment of a case of calciphylaxis in a renal transplant recipient with normal allograft function.


Assuntos
Calciofilaxia , Transplante de Rim/efeitos adversos , Úlcera da Perna , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/patologia , Calciofilaxia/terapia , Humanos , Oxigenação Hiperbárica , Falência Renal Crônica/cirurgia , Perna (Membro)/patologia , Úlcera da Perna/diagnóstico , Úlcera da Perna/etiologia , Úlcera da Perna/patologia , Úlcera da Perna/terapia , Masculino , Pessoa de Meia-Idade , Tiossulfatos/uso terapêutico
16.
Dermatology ; 235(2): 120-129, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30605905

RESUMO

BACKGROUND: Calciphylaxis (CPX) is a rare and life-threatening disease characterized by vascular calcification and development of painful and necrotizing skin lesions with a challenging management. Mechanisms of CPX are complex and include an imbalance between vascular calcification promoters and inhibitors, and frequently vitamin K deficiency. OBJECTIVES: To describe the various presentations and identify predictive factors of death in patients with CPX. METHODS: In this multicenter retrospective study, we included 71 CPX patients followed in South-West France (n = 26) and in French Polynesia (n = 45), and who all received sodium thiosulfate (25 g thrice weekly for a median of 61 days). RESULTS: Characteristics at presentation significantly differed between metropolitan and Polynesian French patients. Polynesians were less frequently on regular dialysis at the onset of CPX, had a higher incidence of diabetes mellitus and obesity, more disturbances of calcium-phosphorus metabolism, and received vitamin K antagonists less frequently than patients from South-West France. Despite intensive management, the 1-year mortality rate was 66% and median time to death was 200 days (IQR, 40; 514). The number of body areas involved (i.e., three: OR 2.70 [1.09; 6.65], p = 0.031; four: OR 8.79 [1.54; 50.29], p = 0.015) was the only predictive factor for death, whereas application of topical cerium nitrate-silver sulfadiazine was protective (OR 0.44 [0.20; 0.99], p = 0.046). Surgical debridement, hyperbaric oxygenation therapy, and geographical origin were not associated with overall outcomes. CONCLUSIONS: Cerium nitrate may lead to vascular decalcification and chelation of reactive oxygen species, and prevent infection. Cerium nitrate-silver sulfadiazine was associated with better outcomes and should be tested in a prospective comparative trial in CPX patients.


Assuntos
Anti-Infecciosos Locais/uso terapêutico , Calciofilaxia/terapia , Cério/uso terapêutico , Sulfadiazina de Prata/uso terapêutico , Dermatopatias Vasculares/tratamento farmacológico , Administração Cutânea , Idoso , Anti-Infecciosos Locais/administração & dosagem , Calciofilaxia/etiologia , Cério/administração & dosagem , Quelantes , Combinação de Medicamentos , Feminino , França , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Polinésia , Diálise Renal , Estudos Retrospectivos , Fatores de Risco , Sulfadiazina de Prata/administração & dosagem , Dermatopatias Vasculares/etiologia , Taxa de Sobrevida , Tiossulfatos/uso terapêutico , Resultado do Tratamento
17.
Clin Dermatol ; 37(5): 528-547, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31896408

RESUMO

The color purple can be seen in several types of eruptions including inflammatory dermatoses like lichen planus, infectious dermatoses like ecthyma gangrenosum, neoplasms like Kaposi sarcoma, and vasculitis and vasculopathy. The current review focuses on the clinical appearance, pathophysiology, and treatment of several vasculitides and vasculopathies including capillaritis, cutaneous small-vessel vasculitis, immunoglobulin A (IgA) vasculitis, cryoglobulinemia, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosum, warfarin-induced skin necrosis, heparin-induced thrombocytopenia, purpura fulminans, antiphospholipid antibody syndrome, calciphylaxis, levamisole-induced vasculopathy, and thrombotic thrombocytopenic purpura. Dermatologists play a central role in treating patients with cutaneous vasculitis and vasculopathy and may have the opportunity to facilitate identification of systemic disease by diagnosing cutaneous vasculitis and vasculopathy.


Assuntos
Pele/patologia , Vasculite/etiologia , Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/etiologia , Calciofilaxia/diagnóstico , Calciofilaxia/etiologia , Calciofilaxia/terapia , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/etiologia , Cor , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/etiologia , Diagnóstico Diferencial , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/etiologia , Granulomatose com Poliangiite/patologia , Humanos , Levamisol/efeitos adversos , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/etiologia , Necrose/induzido quimicamente , Necrose/diagnóstico , Necrose/terapia , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/tratamento farmacológico , Poliarterite Nodosa/etiologia , Púrpura Fulminante/diagnóstico , Púrpura Fulminante/etiologia , Púrpura Fulminante/terapia , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/etiologia , Púrpura Trombocitopênica Trombótica/terapia , Vasculite/diagnóstico , Vasculite/terapia , Varfarina/efeitos adversos
19.
Burns ; 45(1): 241-246, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30322738

RESUMO

Calciphylaxis is a rare, necrotizing skin disorder usually associated with kidney disease, but also caused by many other systemic illnesses. This disease is associated with mortality rates as high as 80% at 1year. We present the demographic and clinical data of nine patients with calciphylaxis treated at our burn center over a 10year period. We review the literature on the clinical presentation, pathophysiology, diagnosis and treatment of this rare disease. We propose that these patients be treated similar to patients with thermal burn injury; meaning intensive wound care, surgical management, critical care and physical therapy. Burn centers are uniquely capable of caring for these incredibly complex patients due to their experience in managing patients with extensive skin and soft tissue defects, wounds and diseases.


Assuntos
Analgésicos/uso terapêutico , Antibacterianos/uso terapêutico , Unidades de Queimados , Calciofilaxia/terapia , Desbridamento , Falência Renal Crônica/terapia , Diálise Renal , Adulto , Idoso , Calciofilaxia/etiologia , Gerenciamento Clínico , Feminino , Humanos , Falência Renal Crônica/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
20.
QJM ; 112(1): 29-34, 2019 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-30304522

RESUMO

Calciphylaxis is a disease of significant morbidity and mortality, predominantly affecting dialysis patients. The term 'calciphylaxis' was coined by Seyle et al. in 1961 to describe calcium deposition in the skin and subcutaneous soft tissue of uremic rats in response to 'triggers' (e.g. trauma, metallic salts) after exposure to 'sensitizing agents' (e.g. vitamin D and parathyroid hormone). In humans, calciphylaxis, however, is not a disorder of induced hypersensitivity. Instead, it is a disorder of cutaneous microvascular occlusion caused by thrombosis and calcification. Progressive, excruciatingly painful, non-healing wounds develop in these patients, pre-disposing them to high risk of sepsis and death. Calciphylaxis has no approved therapies. Increased awareness and research in this field have facilitated identification of risk factors and causation pathways. Development of therapeutic options and wound care management, however, are still at a nascent stage. Certain therapies have shown a promise that needs evaluation in prospective clinical trials. It is hoped that ongoing research will help us better understand the pathogenesis of this complex disease and develop efficacious treatment options. In this review, we outline the components involved in calciphylaxis diagnosis and treatment.


Assuntos
Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Falência Renal Crônica/complicações , Calcificação Vascular/diagnóstico , Calcificação Vascular/terapia , Calciofilaxia/etiologia , Humanos , Falência Renal Crônica/terapia , Diálise Renal/efeitos adversos , Fatores de Risco , Calcificação Vascular/etiologia
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