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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 2  |  Page : 131-132

Novel presentation of harlequin phenomenon (simultaneous cyanotic mottling and frightening pallor [vasospasm] of one limb)


Department of Pediatrics, Bhaktivedanta Hospital and Research Institute, Thane, Maharashtra, India

Date of Web Publication25-Apr-2019

Correspondence Address:
Dr. Sandeep Garg
402/351, Kalpataru, Srishti Sector 3, Mira Road, Thane - 401 107, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.JCN_129_18

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  Abstract 


Harlequin phenomenon is a benign, idiopathic, and rapidly autoresolutive skin phenomenon (discoloration of unilateral body), with no need for treatment. A 2 h-old preterm newborn manifested net-like cyanosis of the left half of the body, followed by severe pallor of the left leg. It normalized in 15 min without any significant intervention. Correct identification of this frightening but transient benign condition and its variants is very important so as to reassure parents and to avoid additional diagnostic procedures and unnecessary treatments.

Keywords: Cyanosis, Harlequin, pallor, vasospasm


How to cite this article:
Garg S, Joshi A, Mehta S. Novel presentation of harlequin phenomenon (simultaneous cyanotic mottling and frightening pallor [vasospasm] of one limb). J Clin Neonatol 2019;8:131-2

How to cite this URL:
Garg S, Joshi A, Mehta S. Novel presentation of harlequin phenomenon (simultaneous cyanotic mottling and frightening pallor [vasospasm] of one limb). J Clin Neonatol [serial online] 2019 [cited 2019 May 22];8:131-2. Available from: http://www.jcnonweb.com/text.asp?2019/8/2/131/257135




  Introduction Top


Harlequin phenomenon is a benign, idiopathic, and rapidly autoresolutive skin phenomenon, with no need for treatment.[1] It occurs occasionally in healthy newborns,[2] characterized by transient color changes in the unilateral half of the body, sharply demarcated at the body's midline.[3] This is probably due to a sympathetic autonomic dysfunction in the control of peripheral capillary bed tonus, probably because of the hypothalamic functional immaturity in the newborn.[1],[4]


  Case Report Top


We describe the case of a preterm (35 weeks old) female child with birth weight of 2.6 kg. She was born through emergency cesarean section in view of abruptio placentae and bleeding per vaginum. The baby was limp, cried after stimulation, and tone improved later on. The baby was shifted to the neonatal intensive care unit in view of grunting respiration and put on oxygen. The baby was started on intravenous (IV) fluid, and breathing and saturation normalized after 1 h.

After 2 h of birth, gradually, bluish-purplish, net-like cyanotic pattern started appearing over the left half of the chest and abdomen and later progressed to the left upper limb and left lower limb [Figure 1]a. This discoloration involved the left side of the body, sparing face and scrotum, most intense on hands, and had clear-cut midline demarcation. It intensified, remained for 15 min, and started fading away. Before it vanished off completely, the left foot turned completely white (severely pale) and progressively involved the left leg up to the knee in caudocephalic direction. It looked as if it has been covered with a white glove [Figure 1]b. This horrifying pattern appeared as if the limb has lost blood supply and going to go in gangrene soon. The pulsations and temperature of the affected part were comparable to those of the opposite limb. This event has alerted the whole team, and this unusual manifestation was discussed with senior neonatologist and dermatologist, and measures to increase perfusion (i.e., warming the limb and giving boluses) were ensued. Within 15 min, before we could start IV nitroglycerin and get vascular surgeon for thrombolytic therapy, the limb color started improving spontaneously in cephalocaudal direction (from the knee toward the foot) and normalized completely within another 15 min, with a great sigh of relief to the whole team.
Figure 1: (a) After 2 h of birth, cyanotic mottling over the left half of the body appeared gradually. (b) Cyanotic mottling vanished within 30 min and the left foot became severely pallor (normalized within 15 min)

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The transient nature and strict unilateral distribution of manifestations confirmed no other etiology other than Harlequin phenomenon.


  Discussion Top


Typically, Harlequin phenomenon presents as a transient, well-demarcated unilateral erythema on the dependent side and pallor on the other half, sometimes sparing arms, legs, trunk, face, and/or genitalia.[5] Although our case had net-like cyanotic pattern on the left side of the body (nondependent) with deep cyanosis of the hands and severe pallor of the foot (extending up to the knee) sparing the face and scrotum (which has not been reported yet), only one case report is available describing severe transient pallor of limb due to Harlequin phenomenon.[6] This variant can be explained by overactivity of left-sided sympathetic nervous system causing cyanotic pattern, being excessively active in the left leg causing severe vasospasm and pallor. Previously, it has been reported with low birth weight, prematurity, hypoxia, systemic use of prostaglandin E1, or intracranial injury.[7] Our case was premature and also had transient hypoxia.

Usually occurring between 2 and 5 days of age, Harlequin color change has been observed as late as 3 weeks of age.[7] The condition is benign, and the change of color fades away in 30 s to 20 min. It may recur when the infant is placed on her or his side. The differential diagnosis includes port-wine stain and nascent hemangioma of infancy.[8] The transient nature of Harlequin phenomenon helps to differentiate it from other conditions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for their child's images and other clinical information to be reported in the journal. The patient's parents understand that their child's name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Valerio E, Barlotta A, Lorenzon E, Antonazzo L, Cutrone M. Harlequin color change: Neonatal case series and brief literature review. AJP Rep 2015;5:e73-6.  Back to cited text no. 1
    
2.
Selimoǧlu MA, Dilmen U, Karakelleoǧlu C, Bitlisli H, Tunnessen WW Jr. Picture of the month. Harlequin color change. Arch Pediatr Adolesc Med 1995;149:1171-2.  Back to cited text no. 2
    
3.
Januário G, Salgado M. The harlequin phenomenon. J Eur Acad Dermatol Venereol 2011;25:1381-4.  Back to cited text no. 3
    
4.
Neligan GA, Strang LB. A “Harlequin” colour change in the newborn. Lancet 1952;2:1005-7.  Back to cited text no. 4
    
5.
Lucky AW. Transient benign cutaneous lesions in the newborn. In: Eichenfield LF, Frieden IJ, Esterly NB, editors. Neonatal Dermatology. 2nd ed. Philadelphia, PA: Saunders Elsevier; 2008. p. 85-97.  Back to cited text no. 5
    
6.
Santos SF, Cancella de Abreu MR. Harlequin phenomenon at birth: And she was born with a white stocking. BMJ Case Rep 2015;2015:bcr2015210549.  Back to cited text no. 6
    
7.
Rao J, Campbell ME, Krol A. The harlequin color change and association with prostaglandin E1. Pediatr Dermatol 2004;21:573-6.  Back to cited text no. 7
    
8.
Cordoro KM, Speetzen LS, Koerper MA, Frieden IJ. Physiologic changes in vascular birthmarks during early infancy: Mechanisms and clinical implications. J Am Acad Dermatol 2009;60:669-75.  Back to cited text no. 8
    


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