Potentially life-threatening emergency
Neonatal purpura fulminans
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Synopsis
Neonatal purpura fulminans is caused by a congenital or acquired absence of protein C and/or protein S that results in a coagulopathy. Rare cases have been associated with antithrombin III deficiency.
Congenital protein C deficiency is caused by a mutation in the PROC gene. Homozygous deficiency is extremely rare and is fatal if not treated. Patients with heterozygous PC deficiency tend to be asymptomatic. However, they are at increased risk of developing purpura fulminans in the setting of increased protein C consumption, such as an infection.
Infectious or idiopathic purpura fulminans can occur in association with or after neonatal infections. Group B streptococcal and gram-negative infections are associated with acquired protein C deficiency in neonates, whereas Neisseria meningitidis and varicella are more common in the adolescent and adult populations. Rare cases of idiopathic purpura fulminans have been reported in infants. See purpura fulminans for further discussion.
Codes
ICD10CM:P60 – Disseminated intravascular coagulation of newborn
SNOMEDCT:
402851000 – Neonatal purpura fulminans (homozygous protein C deficiency)
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Differential Diagnosis & Pitfalls
Coagulation tests must be performed and citrated plasma samples obtained from the patient prior to the initiation of factor replacement or anticoagulation therapy for accurate diagnosis. It may be hard to differentiate congenital and acquired forms of both protein C and S 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; the 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.
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Last Reviewed:12/19/2022
Last Updated:01/11/2023
Last Updated:01/11/2023
Potentially life-threatening emergency
Neonatal purpura fulminans