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Chest clinic
Images in thorax
The Harlequin sign
Free
  1. Duneesha De Fonseka1,
  2. Adriana Lama-Lopez2,
  3. Tim Batchelor3,
  4. Anthony Edey4,
  5. Nicholas A Maskell5
  1. 1Academic Respiratory Unit, North Bristol NHS Trust, Bristol, UK
  2. 2Department of Respiratory, North Bristol NHS Trust, Bristol, UK
  3. 3Department of Thoracic Surgery, Bristol Royal Infirmary, Upper Maudlin Street, Bristol UK
  4. 4Department of Radiology, North Bristol NHS Trust, Bristol, UK
  5. 5Academic Respiratory Unit, Department of Clinical Sciences, Bristol University, Bristol, UK
  1. Correspondence to Dr D De Fonseka, Academic Respiratory Unit, North Bristol NHS Trust, Bristol, BS10 5NB, UK; Duneesha{at}gmail.com

https://doi.org/10.1136/thoraxjnl-2015-206936

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    A 28-year-old woman was incidentally found to have a large right apical mass (figure 1) on a chest radiograph. She denied having any respiratory symptoms but had noted asymmetric flushing of her face following strenuous exercise (figure 2). On closer questioning she also described hypohydrosis affecting the right side of her face.

    Figure 1
    Figure 1

    Chest radiograph showing a large right apical opacity and evidence of thinning of the right second rib posteriorly.

    Figure 2
    Figure 2

    Well-demarcated unilateral flushing following exercise.

    She had further imaging with an MRI scan that was suggestive of a nerve sheath tumour (figures 3 and 4), which was subsequently resected by thoracic surgery. The histology confirmed a schwannoma.

    Figure 3
    Figure 3

    Axial T2-weighted total spin echo MRI image showing a high signal heterogeneous tumour in right apex.

    Figure 4
    Figure 4

    Post-gadolinium T1-weighted MRI sagittal image demonstrating marked tumoural enhancement.

    The ‘Harlequin sign’ is characterised by asymmetric flushing and sweating of the face, representing localised ipsilateral autonomic dysfunction, due to a cervical sympathetic deficit located at the preganglionic or postganglionic level on the non-flushing side.1 In our case, unilateral facial flushing was caused by tumour compression of the cervical sympathetic chain. Since resection of the tumour, her symptoms have improved significantly.

    Harlequin syndrome is a rare syndrome secondary to autonomic dysfunction resulting in anhydrosis and absent or reduced facial flushing on the affected side. Oculosympathetic paresis maybe present in some cases. Causes of Harlequin syndrome include carotid artery dissection, local trauma and neurotropic viral infections but rarely is idiopathic.2 Traditionally, the side with excessive flushing and sweating was perceived to be pathological, but it is now believed that the excessive flushing and sweating is due to a compensatory over-reaction to regulate heat of the face by the unaffected side of the face.3

    References

    1. ↵
      1. Montigiani A,
      2. Cencetti S,
      3. Bandinelli G, et al
      . The “Harlequin Sign.” Case description and review of the literature. Ann Ital Med Int 1998;13:173–5.
      OpenUrlPubMed
    2. ↵
      1. Breunig Jde A,
      2. Hartmann M,
      3. Freire CF, et al
      . Harlequin syndrome in childhood––case report. An Bras Dermatol 2012;87:907–9. doi:10.1590/S0365-05962012000600015
      OpenUrlCrossRefPubMed
    3. ↵
      1. Willaert WI,
      2. Scheltinga MR,
      3. Steenhuisen SF, et al
      . Harlequin syndrome: two new cases and a management proposal. Acta Neurol Belg 2009;109:214–20.
      OpenUrlPubMedWeb of Science
    View Abstract

    Footnotes

    • Collaborator Dr Hilary Archer, Clinical Lecturer in Neurology, University of Bristol.

    • Contributors DDF, AL-L, TB and NAM cared for the patient and wrote the paper. AE reviewed the radiology and critically appraised the paper.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; internally peer reviewed.

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