ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (2)
Potentially life-threatening emergency
Neonatal purpura fulminans
Print
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Neonatal purpura fulminans

Print Images (2)
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.

For information on purpura fulminans due to protein C deficiency, see OMIM.

For information on purpura fulminans due to protein S deficiency, see OMIM.

Codes

ICD10CM:
P60 – Disseminated intravascular coagulation of newborn

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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

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

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 10/06/2017
Last Updated: 10/06/2017
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Neonatal purpura fulminans
Print 2 Images
View all Images (2)
(with subscription)
Neonatal purpura fulminans : Chills, Fever, Gangrene, Ecchymosis, HR increased, BP decreased, PLT decreased, Black eschar
Copyright © 2019 VisualDx®. All rights reserved.