Assuntos
Infecções por Citomegalovirus , Citomegalovirus/metabolismo , Herpesvirus Humano 3/metabolismo , Proteína S/metabolismo , Púrpura Fulminante , Infecção pelo Vírus da Varicela-Zoster , Adulto , Infecções por Citomegalovirus/sangue , Infecções por Citomegalovirus/patologia , Infecções por Citomegalovirus/terapia , Infecções por Citomegalovirus/virologia , Feminino , Humanos , Púrpura Fulminante/sangue , Púrpura Fulminante/patologia , Púrpura Fulminante/terapia , Púrpura Fulminante/virologia , Infecção pelo Vírus da Varicela-Zoster/sangue , Infecção pelo Vírus da Varicela-Zoster/patologia , Infecção pelo Vírus da Varicela-Zoster/terapia , Infecção pelo Vírus da Varicela-Zoster/virologiaRESUMO
BACKGROUND: Purpura fulminans (PF) is a rare, life-threatening condition involving consumptive coagulopathy and intravascular thrombosis, causing purpura and necrosis in the skin and soft tissue. CASE REPORT: A 4-year-old Tajik girl with PF secondary to varicella-zoster virus (VZV) infection presented with purplish red, diffuse, painful lesions localized to the entire right leg. Her vaccination status was unknown, and she did not have concurrent chronic illness. Ten days before admission, the girl was admitted to another hospital in Tajikistan with a diagnosis of chickenpox and PF. She was then transferred to the hospital of the authors of the current report due to the enlargement of lesions to the gluteal region, a change in the color of lesions from red to black, and the detection of arterial thrombosis via Doppler ultrasonography. Multiple surgical debridements were performed to manage tissue necrosis, and the patient's right leg was amputated at the 18th week of admission. The patient was discharged after 26 weeks of hospitalization. CONCLUSION: Although VZV infections mostly cause mild and self-limiting eruptive disease, they can progress, with life-threatening complications, including PF. To prevent VZV infection and resulting complications, immunization with live attenuated vaccines and maintaining population immunity above a certain threshold are the most important strategies to prevent the circulation of the virus.
Assuntos
Púrpura Fulminante , Infecção pelo Vírus da Varicela-Zoster , Humanos , Feminino , Púrpura Fulminante/virologia , Púrpura Fulminante/patologia , Pré-Escolar , Infecção pelo Vírus da Varicela-Zoster/complicações , Varicela/complicações , Desbridamento , Resultado do Tratamento , Amputação Cirúrgica , Herpesvirus Humano 3RESUMO
Nonbacterial purpura fulminans (PF) is rare, usually follows viral infection in young children, and is characterized by specific coagulation disorders, requiring specific therapy. Following a transient rash, a 2-year-old previously healthy girl developed PF without haemodynamic impairment. Laboratory data revealed disseminated intravascular coagulation and a severe transient protein S deficiency. Antiprotein S autoantibodies and active human herpesvirus-6 (HHV6) replication were demonstrated. Purpuric skin lesions spread very rapidly despite broad-spectrum antibiotics and right leg amputation. Plasmapheresis and intravenous immunoglobulins gave complete clinical recovery and normalization of protein S level within 10 days, with progressive clearance of antiprotein S autoantibodies. Transient severe protein S deficiencies have previously been reported in patients with nonbacterial PF, usually after varicella infection. This is the first documented case of PF after HHV6 infection.
Assuntos
Doenças Autoimunes/complicações , Púrpura Fulminante/virologia , Infecções por Roseolovirus/complicações , Amputação Cirúrgica , Doenças Autoimunes/terapia , Pré-Escolar , Coagulação Intravascular Disseminada/etiologia , Feminino , Heparina/uso terapêutico , Herpesvirus Humano 6/isolamento & purificação , Herpesvirus Humano 6/fisiologia , Humanos , Imunoglobulinas/uso terapêutico , Perna (Membro)/cirurgia , Plasmaferese/métodos , Reação em Cadeia da Polimerase , Proteína S/análise , Deficiência de Proteína S/etiologia , Deficiência de Proteína S/terapia , Púrpura Fulminante/terapia , Resultado do Tratamento , Replicação ViralRESUMO
Varicella-associated purpura fulminans is a rare syndrome associated with substantial morbidity and mortality. General supportive care, heparinization, and plasma infusions are the mainstays of treatment. A patient aged 8 years and 8 months with purpura fulminans and multiple deep vein thromboses after varicella infection because of deficiencies of proteins C and S is presented in this case report.
Assuntos
Varicela/complicações , Deficiência de Proteína C/complicações , Deficiência de Proteína S/complicações , Púrpura Fulminante/etiologia , Trombose Venosa/etiologia , Anticoagulantes/uso terapêutico , Testes de Coagulação Sanguínea , Transfusão de Componentes Sanguíneos , Criança , Glucocorticoides/uso terapêutico , Heparina/uso terapêutico , Humanos , Masculino , Metilprednisolona/uso terapêutico , Nadroparina/uso terapêutico , Deficiência de Proteína C/diagnóstico , Deficiência de Proteína C/terapia , Deficiência de Proteína S/diagnóstico , Deficiência de Proteína S/terapia , Púrpura Fulminante/patologia , Púrpura Fulminante/terapia , Púrpura Fulminante/virologia , Resultado do Tratamento , Ultrassonografia Doppler , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/terapia , Trombose Venosa/virologiaRESUMO
Although varicella is usually a benign disease, some of its complications, such as post-varicella purpura fulminans, can be fatal. Its pathophysiological mechanism is caused by the production of antibodies to protein C and protein S in the coagulation cascade. This could have fatal consequences for those patients with partial deficiency of these proteins that develop disseminated intravascular coagulation. Treatment is symptomatic: fresh frozen plasma to treat protein depletion, antithrombin III and heparinization against thrombus formation, and anti-inflammatory drugs (steroids). However, new therapies, such as prostaglandin E1 IV and prostacyclin, are being introduced.
Assuntos
Varicela/complicações , Púrpura Fulminante/virologia , Amputação Cirúrgica , Pré-Escolar , Feminino , Humanos , Perna (Membro)/cirurgia , Púrpura Fulminante/cirurgiaRESUMO
Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.
Assuntos
Antibacterianos/uso terapêutico , Varicela/complicações , Herpesvirus Humano 3 , Leptospirose/complicações , Leptospirose/tratamento farmacológico , Púrpura Fulminante , Adulto , Criança , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Masculino , Púrpura Fulminante/microbiologia , Púrpura Fulminante/patologia , Púrpura Fulminante/virologia , Úlcera Cutânea/patologia , Úlcera Cutânea/cirurgiaRESUMO
The mechanism of postvaricella purpura fulminans is thought to be due to development of antiprotein S antibodies. These antibodies lead to an acquired transient severe protein S deficiency which results in disseminated intravascular coagulation and microvascular thrombosis. Here a patient presented with postvaricella purpura fulminans, where no clinical or laboratory evidence of disseminated intravascular coagulation could be found and there was no deficiency of protein S.
Assuntos
Varicela/complicações , Púrpura Fulminante/virologia , Adulto , Feminino , Gangrena , Humanos , Púrpura Fulminante/tratamento farmacológico , Púrpura Fulminante/patologia , Dedos do PéRESUMO
Varicella-associated purpura fulminans (PF) is a rare complication to varicella infection. The condition is due to autoantibodies directed against protein S which forms part of the anticoagulation system. Lack of protein S leads to disseminated intravascular coagulation in the small vessels, which causes thrombosis and ischemia. Despite early treatment, amputation and skin-grafting is often necessary. In this case story, we give a brief review of the pathogenesis and possible modes of treatment. Knowledge of PF is necessary since early treatment may be life-saving.
Assuntos
Púrpura Fulminante , Autoanticorpos/sangue , Varicela/complicações , Varicela/imunologia , Pré-Escolar , Humanos , Masculino , Deficiência de Proteína S/complicações , Deficiência de Proteína S/imunologia , Púrpura Fulminante/imunologia , Púrpura Fulminante/terapia , Púrpura Fulminante/virologiaRESUMO
Multiorganic failure is a rare manifestation of hantavirus infection but it should be included among differential diagnoses of multiorganic failure in the pediatric age. Currently, there is no effective therapy for this infection. A high suspicion index and early referral to a pediatric intensive care unit with extracorporeal membrane oxygenation may lead to a favourable impact in the outcome. We report a fatal case of hantavirus infection in a 9-year-old girl with a severe disease refractory to hemodynamic supportive measures, and characterized by purpura fulminans, cerebral infarcts, cardiopulmonary failure, and acute renal failure. Commentaries and review about these infrequent clinical manifestations are made.