Purpura fulminans

Seven children with purpura fulminans are reported. All were very sick and had extensive purpuric and ecchymotic skin lesions. Five of them had septicemia, one had meningitis and one had viral respiratory tract infection. The bacteria cultured were Staphylococcus aureus (1), Hemophilus influenzae (1), Pseudomonas aeroginosa (1), Klebsiella pneumoniae (1) and Neisseria meningitidis (2). Consumptive coagulopathy was present in all. Three of the seven children survived. Of the remaining four, two had fulminant meningococcemia and died within 4–5 hours of admission as also a third child with Kleb. pneumoniae septicemia. Two of the three survivors had received heparin as well as steroids apart from treatment of the infection.     

Purpura fulminans bei einem 19 monatigen Kinde

Zusammenfassung Ein Kind mit positivem Wassermann starb innerhalb von 2 Tagen an einer Purpura fulminans.Mit 2 Textabbildungen.     

Purpura fulminans: recognition, diagnosis and management

Purpura fulminans (PF) is a haematological emergency in which there is skin necrosis and disseminated intravascular coagulation. This may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. PF may complicate severe sepsis or may occur as an autoimmune response to otherwise benign childhood infections. PF may also be the presenting symptom of severe heritable deficiency of the natural anticoagulants protein C or protein S. Early recognition and treatment of PF is essential to reduce mortality and to prevent major long-term health sequelae. However, management strategies require accurate identification of the underlying cause. This review focuses on the clinical features, differential diagnosis and laboratory features of the range of PF disorders and includes expert consensus opinion about immediate and on-going management.     

Purpura fulminans in a newborn infant with galactosemia

An 11-day-old neonate presented with purpura fulminans and was subsequently diagnosed with galactosemia. Neonatal purpura fulminans occurs predominantly in patients suffering from inherited protein C deficiency or disseminated intravascular coagulation associated with septicemia. Hemostatic changes in patients with liver disease may result in bleeding or, rarely, thrombosis. We suppose that in the present patient, deficiency of protein C, secondary to liver disease, was responsible for the development of purpura fulminans. Treatment consisted of blood and blood products and galactose-free formula. The patient recovered with residual mild psychomotor retardation and the lesions with minimal scarring. In conclusion, galactosemia also should be kept in mind as an uncommon cause of purpura fulminans in newborn infants.     

Purpura fulminans. A case for heparin therapy

A 6.5-year-old boy developed purpura fulminans. He had no evidence of congenital protein C deficiency. He responded readily to heparin therapy with resolution of his coagulopathy. The coagulopathy resumed rapidly after heparin therapy was interrupted to allow for surgical procedures. Despite correction of his coagulopathy with heparin, surgical amputation of his leg was necessary because of inadequate perfusion.     

Meningokokkensepsis und Purpura fulminans Erfolgreiche Lysetherapie mit rekombinantem Gewebeplasminogenaktivator

Summary A case of a 16 years old young man with meningococcemia, septic shock and purpura fulminans is presented. The clinical course was complicated by ischemia of both lower legs and feet as well as both cheeks. After successful restoration of circulation, the lower legs remained ischemic and loss was imminent. Therefore, fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was started: Both lower legs and feet could be preserved. All of the toes of the right foot, however, had to be amputated. Discussion: Fibrinolytic therapy with rt-PA should be considered as a therapeutic option in patients with meningococcemia and purpura fulminans with imminent loss of extremities. Optimal perfusion pressure and control of coagulation seems to be a prerequisite for successful fibrinolytic therapy. Care should be taken to avoid complications, especially intracerebral hemorrhage.      

Purpura fulminans in a case of protein C deficiency

We report a case of protein C deficiency which presented with purpura fulminans. The inheritance of protein C deficiency is discussed and the importance of warfarin (Coumadin) treatment in this condition is emphasized.Abbreviations DIC disseminated intravascular coagulation - PT prothrombin time     

Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis

A 16-day-old male infant initially was in septic shock. Following intensive resuscitation, thrombohemorrhagic lesions developed over his extremities, except for the limb with an arterial line maintained by a continuous heparin sodium infusion. Blood and CSF cultures yielded group B beta-hemolytic streptococci. Results of laboratory studies and clinical appearance supported the diagnosis of purpura fulminans (PF). Systemic heparinization was therefore started, and subsequently his condition improved. Because of the distinct difference in limb sparing, we concluded heparin has a beneficial effect on the evolution of PF.