Purpura fulminans is a rare form of
disseminated intravascular coagulation characterized by rapidly progressive purpuric lesions,
hypotension and, in some cases,
fever. In neonates,
purpura fulminans usually develops following deficiency of
anticoagulant protein C or S, although acquired forms have been described. The management of
disseminated intravascular coagulation is still controversial, with some studies finding a positive effect of
anticoagulants and others showing no effect or even a detrimental one. Therefore, at present, management is limited to the treatment of underlying disease and replacement of
clotting factors. Personal experience is reported on the efficacy of
heparin in combination with
antithrombin III in the treatment of
purpura fulminans in two preterm neonates who did not have inherited deficiency of
protein C or S and developed the disease possibly following prolonged labor (36 hours) in the first case, and maternal
neoplasia, in the second. Both neonates presented with widespread cyanotic areas rapidly evolving in purpuric lesions and hemorrhagic
bullae. Laboratory tests (prolonged
prothrombin and partial thromboplastin time,
fibrinogen and
antithrombin III concentrations below normal ranges,
d-dimer highly positive) were consistent with
disseminated intravascular coagulation. In both cases
anticoagulant treatment with
heparin (50 UI/kg in bolus followed by 15 UI/kg/h) and
antithrombin III was associated with resolution of
disseminated intravascular coagulation and prompt amelioration of the purpuric lesions, without apparent side effects.