Postvaricella purpura fulminans

Purpura fulminans (PF) is an infrequent complication of varicella characterized by the progressive development of purpuric or painful ecchymotic lesions associated with biochemical alternations typical of consumption coagulopathy. Activation of coagulation is due to a marked and prolonged decrease in protein S, which is probably secondary to the formation of antiprotein S antibodies. The mechanism responsible for the synthesis of these autoantibodies is unknown. We present three cases of postvaricella PF and review the clinical and biochemical characteristics of this entity, as well as current diagnostic and therapeutic recommendations.     

暴发性紫癜的治疗进展

暴发性紫癜(purpura fulminans,PF)征候群又名坏疽性紫癜、坏死性紫癜、出血性紫癜,系儿科危重症,主要为广泛血管内血栓形成,临床表现酷似弥漫性血管内凝血(DIC)。其临床特征为:突然迅速进展的对称性皮肤紫癜,累及全身皮肤,以下肢密集,与其他暴发性皮肤损伤不同的是皮疹可在几小时内由瘀点迅速增大融合为直径为数厘米的瘀斑,基底肿胀坚硬与周围组织分界清楚,颜色由鲜红渐变为暗紫色,坏死后成为黑色焦痂,浆液坏死区发生水泡或血泡,可融合成大泡,发疹的肢体可出现明显肿胀疼痛[1]。本病病因不明,可发生于以下3种情况:急性感染引起的急性感染…     

Diagnosis and management of neonatal purpura fulminans

Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated. The clinical presentation is that of acute disseminated intravascular coagulation and hemorrhagic skin necrosis. The management includes an acute phase of replacement therapy with fresh frozen plasma or protein C concentrate and a maintenance therapy that includes anticoagulation with warfarin or low molecular weight heparin. This review focuses on the management of severe protein C deficiency.     

Idiopathic purpura fulminans with transient protein S deficiency

Idiopathic purpura fulminans produces rapidly progressive hemorrhagic necrosis of the skin with disseminated intravascular coagulation in individuals without known abnormalities of the protein C pathway or acute infections. The disease mainly affects children and in 90 % of cases is preceded by a benign infection. Its pathogenesis involves a temporary autoimmune protein S deficiency that provokes a state of hypercoagulability. We present the case of a previously healthy 2-year-old boy with hemorrhagic skin lesions characteristic of purpura fulminans and disseminated intravascular coagulation without sepsis. Severe, temporary protein S deficiency was confirmed. The patient received daily replacement therapy with fresh frozen plasma for 12 days and anticoagulation with heparin for 3 months. Evolution was favorable. Although the other parameters returned to normal, protein S remained low for 50 days despite treatment. The patient has made a complete recovery.     

Meningococcal purpura fulminans treated with medicinal leeches.

INTRODUCTION: Meningococcal septicemia remains one of the most common infectious causes of admission to a pediatric intensive care unit. Numerous treatment strategies aimed at the thromboembolic complications inducing purpura fulminans and limb/digital ischemia have been attempted, with variable results. The successful use of medicinal leeches for pneumococcal purpura fulminans has been described, and we present a similar case of meningococcal purpura fulminans. PATIENT AND INTERVENTION: A 5-wk-old female infant with meningococcal meningitis and septicemia and progressive purpura fulminans of the left hand was treated with medicinal leeches. Medicinal leeches were applied to the left dorsal hand on a daily basis for 4 consecutive days. RESULT: The swelling and limited functionality visibly improved after 48 hrs, and by 120 hrs, perfusion in the distal phalanges of the thumb and middle finger was evident. Reperfusion of the distal phalanges was not fully sustained, and at 6 wks the plastic surgery department debrided the distal phalanges of her left hand, excluding the thumb. She fully recovered from the meningococcal septicemic shock; the functionality of her left thumb was preserved, and she has limited functionality of her left hand. CONCLUSION: The unique combination of salivary products in leech therapy has theoretical benefits and requires future study.     

High-dose heparin in a patient with purpura fulminans following varicella

We report on a 3 1/2 year old girl with purpura fulminans after varicella who made a complete recovery on treatment with an appropriate high-dose heparin regimen and other supporting measures. Several attempts to reduce the heparin dosage from the initial high level (85 IU/kg/hour) to the usual level (25 IU/kg/hour) were followed each time by an immediate deterioration in the clinical condition.     

A novel protein C mutation causing neonatal purpura fulminans

Background

Neonatal purpura fulminans due to congenital protein C deficiency is a rare disorder.

Case characteristics

A four-day-old neonate presented with multiple necrotic skin lesions with abnormal coagulation profile.

Intervention and outcome

Skin lesions responded to repeated plasma transfusions but the neonate developed bilateral retinal detachment. A novel homozygous PROC gene mutation was noted in the neonate.

Message

Molecular diagnosis and prenatal counseling in neonatal purpura fulminans are vital considering the poor outcome.

     

Neonatal purpura fulminans due to homozygous protein C deficiency

Severe and recurrent purpura fulminans developed in a Turkish boy at 1 week of age. Initial coagulation studies performed were compatible with disseminated intravascular coagulation. Subsequent investigations showed that the patient had homozygous and his healthy parents had heterozygous protein C deficiency. The episodes of purpura fulminans were controlled by infusions of fresh frozen plasma and heparinization. Oral anticoagulant therapy was given in the symptom-free period.     

Epidemiological and prognostic aspects of purpura fulminans in children. Regional survey

A regional survey (Rh?ne-Alpes region) about fulminans purpura has been performed in the pediatric services and the pediatric intensive care units. Between January 1982 and July 1986, 101 cases have been observed. 93.5% (72/77) of the bacteriologically documented cases were due to Neisseria meningitidis. Fatality rate is 34.6%, 58.6% in infants younger than 1 year to 14.3% in children older than 2 years. Between 1982 and 1985 fatality rate was reduced from 54.5% to 27.6%. This study confirms the interest of the severity score established by the French Pediatric Intensive Care Club.     

Management of neonatal purpura fulminans with severe protein C deficiency

Neonatal purpura fulminans is a life threatening clinical entity characterized by extensive subcutaneous thrombosis and disseminated intravascular coagulation usually manifesting shortly after birth. We report an autosomal recessive form of the disease in a neonate who was diagnosed with compound heterozygosity for mutations in his protein C gene as the molecular basis of his disorder.     

Meningococcal purpura fulminans: untoward result of genetic polymorphism?]

Despite significant progress in intensive care medicine, the mortality of septic shock has not changed in recent years. Early recognition of subtle signs in favor of meningococcal sepsis, early antibiotic treatment, and aggressive hemodynamic support remains the cornerstone of therapy of severe meningococcal shock in children. Recent work has emphasized the role of genetic polymorphisms in various systems to explain the most severe cases: anti-inflammatory cytokine profile IL-10/TNF-alpha, elevated levels of plasminogen activator inhibitor type-1, variants of the gene for mannose-binding lectin complement pathway. This may explain the disillusionment of pediatric intensivists, and the general failure of immunotherapy for sepsis. Reasonable hope lies upon new meningococcal vaccines.     

Treatment of acute infectious purpura fulminans with activated protein C

Infectious purpura fulminans is associated with high mortality and morbidity despite standard antimicrobial therapy. We report satisfactory clinical outcome in two children with sepsis associated purpura fulminans who were treated with activated protein C (APC). There is need for proper evaluation of the efficacy of this extremely expensive therapeutic modality by randomized controlled trials before it is made standard of care in childhood infectious purpura fulminans.