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Potentially life-threatening emergency
Neonatal purpura fulminans
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Neonatal purpura fulminans

Contributors: Eric Ingerowski MD, FAAP, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Neonatal purpura fulminans is a rare but life-threatening disorder that presents in a neonate with rapidly progressive intravascular thrombosis and hemorrhagic infarction of the skin. This disorder has high morbidity and mortality rates.

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.

Codes

ICD10CM:
P60 – Disseminated intravascular coagulation of newborn

SNOMEDCT:
402851000 – Neonatal purpura fulminans (homozygous protein C deficiency)

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Diagnostic Pearls

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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.

Best Tests

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Therapy

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References

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Last Reviewed:10/05/2017
Last Updated:01/20/2022
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Potentially life-threatening emergency
Neonatal purpura fulminans
A medical illustration showing key findings of Neonatal purpura fulminans : Chills, Fever, Gangrene, Ecchymosis, HR increased, BP decreased, PLT decreased, Black eschar
Copyright © 2022 VisualDx®. All rights reserved.