Purpura fulminans in the newborn. Report of two cases successfully treated with heparin and antithrombin III

• Published in: Minerva pediatrica Vol. 49; no. 12; p. 571
• Main Authors: Ficuccilli, F, Iacobini, M, Beglieri, M R, Papoff, P, Mancuso, M, Caradonna, A, Donato, A I, Colarizi, P
• Format: Journal Article
• Language: Italian
• Published: Italy 01.12.1997
• Subjects: Anticoagulants - therapeutic use; Antithrombin III - therapeutic use; Female; Heparin - therapeutic use; Humans; Male; Purpura, Schoenlein-Henoch - drug therapy; Remission Induction; Serine Proteinase Inhibitors - therapeutic use
• ISSN: 0026-4946

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.