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Sarvana Sravya*, Dhondge Sai Sundar and Dr. Mehraj Fatima


Purpura fulminans (PF) is a hematological emergency characterized by skin necrosis and disseminated intra-vascular coagulation. It was first described by Guelliot Hjort et al. were the first to clearly define its characteristic features in 1884. Purpura fulminans is not a single disease, but a common clinical and histopathological manifestation of several distinct disease processes. This syndrome usually occurs in children, but it has also been noted in adults. Neonatal purpura, Idiopathic purpura and Acute infectious purpura are the three basic prototypes of purpura fulminans. This article gives a brief discussion regarding symptoms, causes, pathology, diagnostic tests and treatment of various types of purpura. Purpura fulminans occur due to congenital or adapted factors that alter the anticoagulants level in the body.Protein C deficiency is common, it may be associated with deficiency of other anticoagulants, protein S and antithrombin-III. Purpura shows thrombotic occlusions of small and medium blood vessels in the body leading to extra-vascular bleeding. Symptoms like well-demarked ecchymosis patches/ maculae are seen initially, followed by irregular central blue-black necrotic areas representing cell death. In neonates, the symptoms appear abruptly with-in 2-3 days of birth and rapid progression of condition leads to increased risk of multi- organ failure and mortality. In adults, the progression is slow comparatively. Physical examination of lesions and protein C levels are useful for immediate treatment or preventive measures to be followed. Management strategies are made based on diagnostic results. The general management consists of fresh frozen plasma (FFP); plasma concrete and anticoagulants.

Keywords: purpura, skin necrosis, thrombocytopenia, anticoagulants, thrombosis, Ceprotin.

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