A newborn girl presents with severe Purpura Fulminans. Family has history of 1 year old male died of severe purpura fulminans. Other 2 siblings are normal. What is the diagnosis?
Purpura fulminans: Coagulation of the microvasculature, which leads to purpuric lesions and skin necrosis. =3 types: NEONATAL: Hereditary deficiency of protein C, protein S and antithrombin III IDIOPATHIC: Post infectious autoimmune disorder ACUTE INFECTIOUS: most common type- manifests as a skin finding in the most severe septic patients as well as in necrotizing fascitis SINCE the neonate has severe purpura fulminans and family history- answer is protein C deficiency.
Hemophilia A
Protein C deficiency
ITP
vWD
JIPMER 2018