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Abstract

Protein C (PC) deficiency is a rare but life-threatening bleeding disorder that can present in the immediate neonatal period. This article presents the case of a baby girl with acute and progressive neonatal purpura fulminans as the presenting feature of PC deficiency. Other common complications of this disease include ophthalmic problems and central nervous system (CNS) changes. Management consists of correcting the coagulopathy, intensive wound care including negative-pressure dressings and skin grafting, and supportive care for the ophthalmic and CNS issues. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.

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