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Article: Volume 43 Number 1 Page 90 - January/February 2016

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  Dent Update 2016; 43: 90-92

Oral medicine:  A Case of Undiagnosed Harlequin Syndrome Presenting in General Dental Practice

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Abstract: Harlequin syndrome is a rare, clinically striking syndrome characterized by distinctly demarcated asymmetric facial flushing and sweating. It may be of idiopathic aetiology or caused by demonstrable ipsilateral damage to the sympathetic nervous system.1 A case is described where a patient presented to her general dental practitioner complaining of distinctly demarcated unilateral facial flushing and sweating. Onward referral resulted in a diagnosis of Harlequin syndrome.

Clinical relevance: This article highlights the neurological signs and symptoms of Harlequin syndrome, making it easier to recognize if it presents in general dental practice.

Author notes: Kathryn Edwards, BDS(Hons), MFDS RCS(Ed), School of Dental Sciences, Newcastle University, Andrew Schaefer, MBChB, MRCP(UK), Department of Neurology, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, Mark Greenwood, BDS, MDS(Manc), FDS RCS(Eng), MB, ChB(Leeds), PhD(Newc), FRCS(Eng), FRCS(OMFS), FHEA and Konrad Staines, BChD(Malta), FDS RCS(Eng), MOMed, RCS(Edin), School of Dental Sciences, Newcastle University, Framlington Place, Newcastle upon Tyne, NE1 4LP, UK.

Objective: To understand the background of Harlequin syndrome and recognize the need for appropriate specialist referral for definitive diagnosis.