Journal of Dermatology 2014; 41: 102–103
doi: 10.1111/1346-8138.12277
CONCISE COMMUNICATION
Harlequin color change in two preterm newborns Dan DANG, Wenli ZHOU, Ying LIU, Hui WU Department of Neonatology, The First Hospital of Jilin University, Changchun, China
ABSTRACT Harlequin color change is a benign transient skin change usually seen in the neonatal period with unknown etiology. It has been rarely reported in preterm infants. Herein, we report two cases of harlequin color change developed in premature infants without any adverse consequences. During the event, one infant was receiving antibiotic treatment for an Escherichia coli bacteremia in addition to routine supportive care and the other one was in a very stable condition. Both preterm infants were also diagnosed to have a patent ductus arteriosus. As a benign transient skin change, harlequin color change should be recognized properly to avoid unnecessary treatment.
Key words:
harlequin color change, newborn, preterm.
INTRODUCTION
(a)
Harlequin color change is a rare benign skin lesion, observed in young infants, especially newborn. It is a sudden and brief paroxystic change in skin color, distinctly delimited in the midline, bisecting the entire body into a pale and a plethoric half.1 The incidence of harlequin color change was reported to be 8.8% in the initial report in the 1950s2 but there is much incoherence in clinical experience and the published work. We herein report on two preterm neonates with harlequin color change.
CASE 1 A baby girl conceived by in vitro fertilization was born via normal vaginal delivery at 30 weeks of gestation with a birthweight of 1.45 kg. The baby was admitted into our neonatal intensive care unit (NICU) for very low birthweight and respiratory distress syndrome (RDS). The baby was put on mechanical ventilation and was treated with pulmonary surfactant along with parenteral nutrition. After admission, the baby’s condition deteriorated. A small patent ductus arteriosus (PDA) was confirmed by echocardiograph on day 2. The baby’s initial admission blood culture was positive for Escherichia coli. She was treated with meropenem for early onset neonatal sepsis. Approximately 72 h after birth, after a blood drawn from the right upper arm, the infant appeared to have a definite color change consisting of diffuse redness of the left side of body and pallor of the right side of the body with a clear midline demarcation extending from the face to the lower limbs which subsided on its own after a few seconds (Fig. 1a). There were seven similar episodes that were noticed after the first one. One episode was after the baby was placed in the lateral position, one was
(b)
Figure 1. (a) Definite color change consisting of diffuse redness of the left side of the body and pallor of the right side of the body with a clear midline demarcation extending from the face to the lower limbs. (b) Definite color change consisting of diffuse redness of the right side of the body and pallor of the left side of the body with a clear midline demarcation extending from the face to the lower limbs.
Correspondence: Hui Wu, M.D., Ph.D., Department of Neonatology, The First Hospital of Jilin University, 71 Xinmin Street, Changchun 130021, China. Email: [email protected] Received 6 June 2013; accepted 7 August 2013.
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Harlequin color change
after blood was drawn and one was after suctioning. Four other episodes did not have any aggravating factors. Each episode alternated between the left and right side of the body and subsided on their own after a few seconds without any intervention. The infant was discharged after 4 weeks and was followed up to 1 year of age. There were no signs of harlequin color change after discharge home. During the follow up, the growth and development of the infant was normal.
CASE 2 A 31-week baby girl with birthweight of 1.63 kg was born by normal vaginal delivery after a precipitated delivery with delayed ligation of the umbilical cord. The baby was admitted to the NICU after 8 h of birth for prematurity and low birthweight. After admission, the baby was placed on continuous positive airway pressure support and blood was drawn for laboratory examination. It was noticed that the baby had a difficulty in stopping bleeding. The coagulation profile revealed an activated partial thromboplastin time of 108.7 s, prothrombin time of 19.8 s and international normalized ratio of 1.68. Echocardiography showed PDA of 1.5 mm. On day 3 of life, while the infant was in a supine position, a definite color change with a clear line of demarcation appeared extending from the face to the trunk, one side of the body was erythematous and the other side of the body was pale, which lasted for 3 min (Fig. 1b). Within the following 3 days, while the baby was in a supine position, several episodes of the harlequin color change happened again which lasted for 1–3 min each. The capillary refill time and monitoring of the oxygen saturation and the blood pressure of four limbs were all normal. The heart rate and the respiratory rate were stable. The infant was discharged after 3 weeks in the NICU and followed up to 6 months of age. During follow up, no further episode was reported, and the growth and development of the child were normal.
DISCUSSION Harlequin color change is a rarely observed skin lesion, the etiology and pathogenesis of which are still not clear. Some suggests that it may be related to hypothalamic control immaturity of the sympathetic peripheral vascular tonus which causes a sympathetic disautonomy. As a result, unstable vasoconstriction induces unilateral erythema with contralateral pallor.1–7 The redness of the skin was due to the weak sympathetic
© 2013 Japanese Dermatological Association
vasoconstriction leading to vasodilatation of the ipsilateral side. On the contrary, pallor of the contralateral side was due to vasoconstriction. Others incline to take the view that hypoxia causes harlequin color change, as its incidence is high in infants with cyanotic heart disease. Apparently, in monozygotic twins, harlequin color change has happened more often in the one with congenital heart disease, and further has disappeared after the heart disease has been cured.1,3–5,8 To some extent, harlequin color change can be affected by position, and it is easily observed when the subject is lying down on the side.1–3 In our two cases, the presence of RDS, PDA or sepsis may have led to unstable peripheral vasoconstriction causing harlequin color change. In addition, lateral position, blood drawing or suctioning indicates that gravity or pain may be the causative factors. In summary, harlequin color change is a recurrent, benign and self-limiting skin color change condition usually seen in the neonatal period. It lasts for a very short duration and is not associated with significant hemodynamic, respiratory or abnormal neuromuscular changes, which differentiates it from other serious diseases.
CONFLICT OF INTEREST: The authors have no conflicts of interest to declare in relation to this article. REFERENCES 1 Selimoglu MA, Dilmen U, Karakelleoglu C et al. Picture of the month Harlequincolor change. Arch Pediatr Adolesc Med 1995; 149: 1171– 1172. 2 Neligan GA, Strang LB. A “harlequin” colour change in the newborn. Lancet 1952; 260: 1005–1007. 3 Lucky AW. Transient benign cutaneous lesions in the newborn. In: Eichenfield LF, Frieden IJ, Esterly NB, eds. Neonatal Dermatology, 2nd edn. Philadelphia: Saunders Elsevier 2008. 4 Walker NP, Champion RH. Neonatal dermatology. In: Roberton NRC, ed. Textbook of Neonatology, 2nd edn. Edinburgh: Churchill Livingstone, 1992. 5 Morelli JG. Diseases of the neonate. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics, 18th edn. Philadelphiam: Saunders Elsevier, 2007. 6 Pierson HA, Cone TE Jr. Harlequin color change in a young infant with tricuspid atresia. J Pediatr 1957; 50: 609–612. rio G, Salgado M. The Harlequin phenomenon. J Eur Acad Der7 Janua matol Venereol 2011; 25: 1381–1384. 8 Rao J, Campbell ME, Krol A. The harlequin color change and association with prostaglandin E1. Pediatr Dermatol 2004; 21: 573–576.
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doi: 10.1111/1346-8138.12277
CONCISE COMMUNICATION
Harlequin color change in two preterm newborns Dan DANG, Wenli ZHOU, Ying LIU, Hui WU Department of Neonatology, The First Hospital of Jilin University, Changchun, China
ABSTRACT Harlequin color change is a benign transient skin change usually seen in the neonatal period with unknown etiology. It has been rarely reported in preterm infants. Herein, we report two cases of harlequin color change developed in premature infants without any adverse consequences. During the event, one infant was receiving antibiotic treatment for an Escherichia coli bacteremia in addition to routine supportive care and the other one was in a very stable condition. Both preterm infants were also diagnosed to have a patent ductus arteriosus. As a benign transient skin change, harlequin color change should be recognized properly to avoid unnecessary treatment.
Key words:
harlequin color change, newborn, preterm.
INTRODUCTION
(a)
Harlequin color change is a rare benign skin lesion, observed in young infants, especially newborn. It is a sudden and brief paroxystic change in skin color, distinctly delimited in the midline, bisecting the entire body into a pale and a plethoric half.1 The incidence of harlequin color change was reported to be 8.8% in the initial report in the 1950s2 but there is much incoherence in clinical experience and the published work. We herein report on two preterm neonates with harlequin color change.
CASE 1 A baby girl conceived by in vitro fertilization was born via normal vaginal delivery at 30 weeks of gestation with a birthweight of 1.45 kg. The baby was admitted into our neonatal intensive care unit (NICU) for very low birthweight and respiratory distress syndrome (RDS). The baby was put on mechanical ventilation and was treated with pulmonary surfactant along with parenteral nutrition. After admission, the baby’s condition deteriorated. A small patent ductus arteriosus (PDA) was confirmed by echocardiograph on day 2. The baby’s initial admission blood culture was positive for Escherichia coli. She was treated with meropenem for early onset neonatal sepsis. Approximately 72 h after birth, after a blood drawn from the right upper arm, the infant appeared to have a definite color change consisting of diffuse redness of the left side of body and pallor of the right side of the body with a clear midline demarcation extending from the face to the lower limbs which subsided on its own after a few seconds (Fig. 1a). There were seven similar episodes that were noticed after the first one. One episode was after the baby was placed in the lateral position, one was
(b)
Figure 1. (a) Definite color change consisting of diffuse redness of the left side of the body and pallor of the right side of the body with a clear midline demarcation extending from the face to the lower limbs. (b) Definite color change consisting of diffuse redness of the right side of the body and pallor of the left side of the body with a clear midline demarcation extending from the face to the lower limbs.
Correspondence: Hui Wu, M.D., Ph.D., Department of Neonatology, The First Hospital of Jilin University, 71 Xinmin Street, Changchun 130021, China. Email: [email protected] Received 6 June 2013; accepted 7 August 2013.
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© 2013 Japanese Dermatological Association
Harlequin color change
after blood was drawn and one was after suctioning. Four other episodes did not have any aggravating factors. Each episode alternated between the left and right side of the body and subsided on their own after a few seconds without any intervention. The infant was discharged after 4 weeks and was followed up to 1 year of age. There were no signs of harlequin color change after discharge home. During the follow up, the growth and development of the infant was normal.
CASE 2 A 31-week baby girl with birthweight of 1.63 kg was born by normal vaginal delivery after a precipitated delivery with delayed ligation of the umbilical cord. The baby was admitted to the NICU after 8 h of birth for prematurity and low birthweight. After admission, the baby was placed on continuous positive airway pressure support and blood was drawn for laboratory examination. It was noticed that the baby had a difficulty in stopping bleeding. The coagulation profile revealed an activated partial thromboplastin time of 108.7 s, prothrombin time of 19.8 s and international normalized ratio of 1.68. Echocardiography showed PDA of 1.5 mm. On day 3 of life, while the infant was in a supine position, a definite color change with a clear line of demarcation appeared extending from the face to the trunk, one side of the body was erythematous and the other side of the body was pale, which lasted for 3 min (Fig. 1b). Within the following 3 days, while the baby was in a supine position, several episodes of the harlequin color change happened again which lasted for 1–3 min each. The capillary refill time and monitoring of the oxygen saturation and the blood pressure of four limbs were all normal. The heart rate and the respiratory rate were stable. The infant was discharged after 3 weeks in the NICU and followed up to 6 months of age. During follow up, no further episode was reported, and the growth and development of the child were normal.
DISCUSSION Harlequin color change is a rarely observed skin lesion, the etiology and pathogenesis of which are still not clear. Some suggests that it may be related to hypothalamic control immaturity of the sympathetic peripheral vascular tonus which causes a sympathetic disautonomy. As a result, unstable vasoconstriction induces unilateral erythema with contralateral pallor.1–7 The redness of the skin was due to the weak sympathetic
© 2013 Japanese Dermatological Association
vasoconstriction leading to vasodilatation of the ipsilateral side. On the contrary, pallor of the contralateral side was due to vasoconstriction. Others incline to take the view that hypoxia causes harlequin color change, as its incidence is high in infants with cyanotic heart disease. Apparently, in monozygotic twins, harlequin color change has happened more often in the one with congenital heart disease, and further has disappeared after the heart disease has been cured.1,3–5,8 To some extent, harlequin color change can be affected by position, and it is easily observed when the subject is lying down on the side.1–3 In our two cases, the presence of RDS, PDA or sepsis may have led to unstable peripheral vasoconstriction causing harlequin color change. In addition, lateral position, blood drawing or suctioning indicates that gravity or pain may be the causative factors. In summary, harlequin color change is a recurrent, benign and self-limiting skin color change condition usually seen in the neonatal period. It lasts for a very short duration and is not associated with significant hemodynamic, respiratory or abnormal neuromuscular changes, which differentiates it from other serious diseases.
CONFLICT OF INTEREST: The authors have no conflicts of interest to declare in relation to this article. REFERENCES 1 Selimoglu MA, Dilmen U, Karakelleoglu C et al. Picture of the month Harlequincolor change. Arch Pediatr Adolesc Med 1995; 149: 1171– 1172. 2 Neligan GA, Strang LB. A “harlequin” colour change in the newborn. Lancet 1952; 260: 1005–1007. 3 Lucky AW. Transient benign cutaneous lesions in the newborn. In: Eichenfield LF, Frieden IJ, Esterly NB, eds. Neonatal Dermatology, 2nd edn. Philadelphia: Saunders Elsevier 2008. 4 Walker NP, Champion RH. Neonatal dermatology. In: Roberton NRC, ed. Textbook of Neonatology, 2nd edn. Edinburgh: Churchill Livingstone, 1992. 5 Morelli JG. Diseases of the neonate. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics, 18th edn. Philadelphiam: Saunders Elsevier, 2007. 6 Pierson HA, Cone TE Jr. Harlequin color change in a young infant with tricuspid atresia. J Pediatr 1957; 50: 609–612. rio G, Salgado M. The Harlequin phenomenon. J Eur Acad Der7 Janua matol Venereol 2011; 25: 1381–1384. 8 Rao J, Campbell ME, Krol A. The harlequin color change and association with prostaglandin E1. Pediatr Dermatol 2004; 21: 573–576.
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