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Purpura fulminans (PF) is a rare but devastating complication of sepsis characterized by a highly thrombotic subtype of disseminated intravascular coagulation (DIC). A medical emergency, PF cases often require the involvement of consultant hematologists to assist with diagnosis and management of patients who are in a highly dynamic and deteriorating clinical situation. Patients who survive past the first 24 to 72 hours often die from complications of unchecked thrombosis rather than from shock, and survivors are usually left with severe scarring and tissue loss.

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Article Synopsis
  • Neonatal protein C deficiency can lead to severe conditions like purpura fulminans and has a generally poor prognosis, especially regarding vision.
  • In a study of 38 survivors with biallelic PROC variants, a significant number suffered from severe visual impairments: 23 were totally blind, and only one had normal vision.
  • Prenatal intervention is crucial to enhance visual outcomes, as ocular lesions often present early in these cases, affecting the eyes before other organs.
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When an understanding of pathogenesis exists, skin lesions that have the appearance of blood in the skin can provide insight into the mechanisms leading to a systemic process that results in cutaneous manifestations. Of the vascular disturbances of the skin that occur in critically ill patients, some result from a non-hemorrhagic process while occurs represent bleeding into the skin. The lesions of livedo, petechiae, purpura, and ecchymoses can be approached from such a perspective.

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