Harlequin syndrome: A case report in Uyo, southern Nigeria

Harlequin syndrome is a rare autonomic disorder characterized by ipsilateral loss of sweating or flushing of the face, neck and upper thoracic region in response to heat, exercise or emotional stress. It may be primary or secondary and occurs at any age. Very few cases have been reported in Africa and this to the best of our knowledge is the first case report in Nigeria. The patient is a 29 year old cinematographer who presented with complaints of sweating only on the right side of the face, neck and trunk for four months. He first noticed that he was sweating on the right side of his face, neck and trunk while taking pictures and videos outside on an extremely hot day. Since then he had noticed this characteristic sweating. There was associated darkening of the right side of the body. There was no history of trauma to the neck, back or neck surgery. He had no associated gustatory sweating, erectile dysfunction, palpitations, syncopal attacks or nocturnal diarrhoea. Past medical history was not contributory. Examination revealed sweating with associated hyperpigmentation of the right side of the face with anhidrosis of the left side. A clinical diagnosis of Harlequin Syndrome was made. The routine investigations were all normal. Further investigations to exclude secondary causes were hampered by lack of funds. He is regular on follow-up and has no further complaints. Rare diseases still occur and require a high index of suspicion and good clinical acumen to make a diagnosis.