Patient Presentation
The parents of a 9-month-old female sent pictures of their daughter’s face through the institution’s electronic medical record and wanted to know if they should be worried about a rash. The pediatrician noted generalized facial redness on the left half of the face which created a well-demarcated line down the center of the patient’s face. It appeared to stop along the jaw-line and he could not determine if it continued into her scalp or towards her ear. She had no observable eye changes or tearing, swelling of the skin or lips and no perspiration, and the patient was smiling in at least 1 picture. The parents denied any other body areas being affected, any new contacts with soaps/detergents/sunscreen etc., and said she was otherwise well. They said that the redness had subsided without any intervention after several minutes but recurred one other time in the past and again went away. The pediatrician showed the pictures to a colleague and both agreed it looked most like the diagnosis of harlequin skin changes. He recommended to the family to monitor the infant as she was previously well and he would see her in the next couple of weeks for her routine well-child care.
Discussion
Harlequins are comic characters originally from the Italian Commedia dell’arte who wore characteristic masks and brightly colored, well-demarcated (outlined) costumes. The comic’s or clown’s costumes vary and many are depicted with diamond shaped patterns. Colors can be predominantly bi-colored or multi-colored. Some costumes will divide the body in half or quarters with each section in a contrasting color or pattern. Pablo Picasso famously painted and sculpted harlequin performers many times in various costumes. Some examples can be seen here.
There are at least 3 entities in medicine which use the term harlequin.
Harlequin syndrome is a rare autonomic disorder. On the unaffected, or non-pathological half of the face, there is well-demarcated facial flushing which can be intense and the skin perspires. On the affected half, there is absent or reduced flushing and anhidrosis. One case report describes it as occurring bilaterally. It can be induced by heat, exercise or stress. It is mainly acquired but can be congenital (6%) and occurs in all ages. It is usually benign but can be associated with Horner’s syndrome and other similar ocular/facial syndromes, head/neck/brain masses, and viruses which attack the central nervous system. It is a diagnosis of exclusion. It was first described by Land and Drummond in 1988.
Harlequin ichthyosis (sometimes termed Harlequin Baby Syndrome) is an congenital lamellar ichythosis that is autosomal recessive and where the patient has diamond-like, plate-like scales.
Learning Point
Harlequin color change was described in 1952 by Neligan and Strangis. It is a transient sign seen especially in neonates and young children, and is more widely reported than the others above. There is usually a clear demarcation of 1/2 of the face or body, or sometimes alternating body and facial areas, where half turns pink/red and the other side remains normal flesh-colored. The color changes lasts several minutes and then resolves and there is no associated change in perspiration. It is believed to be benign occurring due to changes in vascular tone but the cause is not entirely explained. It can occur after anesthetics or alprostadil but usually occurs without any medication. There is at least 1 study documenting differences in facial temperature without unilateral facial flushing in patients receiving thoracic epidural anesthesia which the authors state may indicate that there is a subclinical harlequin color change.
Questions for Further Discussion
1. How do you explain vasomotor instability?
2. What types of skin changes are often seen in vasomotor instability?
3. What clinical signs constitute Horner syndrome?
4. What causes red skin coloration? A review can be found here
Related Cases
- Disease: Harlequin Skin Changes | Skin Diseases
- Symptom/Presentation: Rash
- Specialty: Dermatology
- Age: Infant
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for this topic: Skin Diseases
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Kang JH, Zafar, MS, Werner KE. Child Neurology: An infant with episodic facial flushing: A rare case and review of congenital harlequin syndrome. Neurology. 2018;91(6).
Rovner MS, Redding AT, Wolf BJ, Wharton JA, Risely CJ, Furse CM. Detection of subclinical Harlequin syndrome in pediatric patients. Pediatr Anesth. 2020;30(5):592-598. doi:10.1111/pan.13852
van den Berg G, Bakker H. Harlequin Color Change in a Neonate. N Engl J Med. 2020;382(5):456-456. doi:10.1056/NEJMicm1907713
Douvali T, Papageorgiou V, Gerochristou M, Gerodimou M, Tampouratzi E, Chasapi V. Harlequin syndrome in a young girl: An opportunity to rethink the terminology. Pediatr Dermatol. 2021;38(4):984-985. doi:10.1111/pde.14681
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa