Neonatal purpura fulminans
Neonatal purpura fulminans can be caused by a congenital absence of protein C and/or protein S that results in dermal microvascular thrombosis that progresses to disseminated intravascular coagulation (DIC), perivascular hemorrhage, and hemorrhagic infarction of the skin with necrosis. Rare cases have also been associated with antithrombin III deficiency.
A similar presentation (often termed idiopathic or infectious purpura fulminans) can occur in association with or after neonatal infections. Meningococcemia is associated with acquired protein C deficiency. Similar presentations have been described with gram-negative infections as well as with staphylococcal and streptococcal (group B) infections, although impact on protein C levels in these cases are variable. Rare cases of idiopathic purpura fulminans have been reported in infants. See purpura fulminans for further discussion.
For information on purpura fulminans due to protein C deficiency, see OMIM.
For information on purpura fulminans due to protein S deficiency, see OMIM.
P60 – Disseminated intravascular coagulation of newborn
402851000 – Neonatal purpura fulminans (homozygous protein C deficiency)
- Necrotizing fasciitis – More localized skin presentation (one area of body).
- Idiopathic thrombocytopenic purpura – Platelet count will be low but there are no signs of DIC; patient may have areas of purpura but no areas of necrosis.
- Thrombotic thrombocytopenic purpura
- Sepsis – Can lead to DIC and skin purpura.
- Acute hemorrhagic edema of infancy – Uncommon to see any systemic or visceral involvement.
- Hypersensitivity vasculitis
- Meningococcemia – Associated with acquired protein C deficiency.
Last Updated: 10/06/2017