Fulminant purpura due to protein S deficiency (non-inflammatory occlusive disease)
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Abstract
It is a rare condition characterized by extensive tissue thrombosis and hemorrhagic skin necrosis. It manifests itself in three clinical situations: in newborns with homozygous congenital deficiency of protein C, protein S, antithrombin III and/or resistance to activated protein C due to factor V Leiden mutation, in severe sepsis, characteristically fulminant meningococcemia; and in the absence of acute infection, associated with a transient autoimmune deficiency of protein S.
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References
De Kleinjn ED, de Groot R, Hack E, Mulder PGH, Eng W, Moritz B, et al. Activation of protein C following infusion of protein C concentrate in children with severe meningococcal sepsis and purpura fulminans: a randomized, double-blinded, placebo-controlled, dose- finding study. Critical care. 2003;31(6):1839-47. doi: 10.1097/01.CCM.0000072121.61120.D8.