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Harlequin color change of newborn
Other Resources UpToDate PubMed

Harlequin color change of newborn

Contributors: Michael Gerbo BS, Elizabeth Duarte MD, William Farmer MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Harlequin color change (HCC) of the newborn, or harlequin phenomenon, is a transient unilateral erythema that is seen in up to 10% of healthy newborns. HCC is macular and blanchable, and displays a sharp midline cutoff. It is often gravity dependent. HCC typically presents between 2 and 5 days of life, with some infants presenting with episodes up to the third week. The exact etiology of HCC is unknown, but current evidence points to sympathetic autonomic dysfunction in the control of capillary tone, likely secondary to hypothalamic immaturity in the newborn.

Most cases are idiopathic in healthy neonates. HCC has also been noted in the setting of prematurity, low birth weight, hypoxia, infusion of prostaglandin E1 (PGE1), and neonatal meningitis. 

The transient color change lasts anywhere from 30 seconds to 20 minutes and recurs up to 12 times over a period of 24 hours. Of note, typical HCC occurs without changes in vital signs, pain, or other signs or symptoms. Hemiscrotal- and hemifacial-limited variants as well as a patchy erythema variant have been reported.

Codes

ICD10CM:
P29.89 – Other specified conditions originating in the perinatal period

SNOMEDCT:
276697006 – Harlequin change

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Cutis marmorata – Transient reddish-purple, reticulated mottling of the skin that involves the trunk and extremities.
  • Cutis marmorata telangiectatica congenita – Reticular erythematous or violaceous patches on one or more extremity, sometimes involving the trunk.
  • Port-wine stain – A fixed partially or completely blanchable pink or red patch, with unilateral or segmental distribution that respects the midline.
  • Nascent hemangioma of infancy – Bright red papule, nodule, or plaque raised above clinically normal skin. Typically not evident at birth, but becomes apparent within the first days to months of life.
  • CHILD syndrome – X-linked dominant disorder characterized by congenital hemidysplasia, ichthyosiform erythroderma, and limb defects. The skin involvement is unilateral, with a sharp midline demarcation, and consists of circumscribed linear plaques covered by waxy scales.
  • Vaso-occlusive complications from central catheters
  • Acrocyanosis
  • Viral infection
  • Drug reaction

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:07/12/2020
Last Updated:07/26/2020
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Harlequin color change of newborn
A medical illustration showing key findings of Harlequin color change of newborn : Developed during first days to week of life, Erythema, Unilateral distribution, Blanching macules
Copyright © 2022 VisualDx®. All rights reserved.