Background Idiopathic unilateral anhidrosis which is also known as Harlequin syndrome was first reported by lance et al. in 1988.It is a rare disorder common in females than males. Usual presentation is unilateral facial anhidrosis with flushing and hyperhidrosis in the opposite side. Anhidrosis can also be involved in arms, neck and trunk
The patient is a 36years old female from Palakkad Kerala. She was admitted for NSAID induced vomiting. No history of headache or sleep disturbance was present. During the hospital stay we noticed hyperhidrosis on left side of her neck. It has been there for around 10years and was not evaluated till then. She is having diabetes mellitus and hypothyroidism since 3years and has been under treatment for the same. She has not undergone any neck surgery. there was no history of postural hypotension, sensory loss or motor weakness.
The clinical examination was normal except for unilateral anhidrosis over face, neck and trunk. No postural hypotension was recorded in our evaluation. Neurological examination did not show any motor or sensory deficit. deep tendon reflexes were intact (ruled out Horner's, syndrome, Adie’s syndrome and Ross syndrome)
Laboratory investigation
In the stress test conducted by asking the patient to race for 10minutes, sweating appeared over the left side of face, neck and trunk with no sweating on the otherside. Her plasma glucose levels were normal. Blood investigations done to screen for HIV, HBSAG, HCV, SERUM ANA, were found to be negative. MRI Brain and spine done to rule out any structural abnormality was normal. bilateral carotid artery doppler showed normal results. Thus, a diagnosis of idiopathic unilateral anhidrosis; Harlequin syndrome was made.
Harlequin syndrome is characterised by sweating and discoloration over one side of the face and anhidrosis on the other side. It is caused by unilateral blockade of sympathetic fibers which carry vasodilator and sudomotor nerves which supply the face. Its primarily idiopathic in around 60% cases. It is commonly triggered by exercise, emotions, spicy food or heat. However, it can also be a manifestation of underlying cervical and uppeethoracic pathologies resulting in the compression of aympathetic fibres or iatrogenic from the surgical or anesthetic procedures around the neck. It is a benign and self-limiting syndrome. But it can cause distress to the patient due to sudden discoloration and sweating over one side of the face following a stimulus. It can also bring distress and anxiety to the patient and family considering the neurological association of the clinical syndrome. There is one case reported in which the patient presented with the cold sensation of left arm. They found out that there was sweating only on left side of face and neck after running on a treadmill at 13km/h. The rarity and variability in presentation pose diagnostic dilemma for clinicians. Females are affected more often than males (66% versus 34%). The flushing was also involved in arm and trunk apart from face in one third of cases. about half of the individuals presented with horners syndrome as well. This case is an example of classical Harlequin syndrome of idiopathic origin which has a benign natural course. The symptoms persist for a longtime without any exacerbation or complication. In individuals with dark complexion flushing could not be appreciated as in our case. One similar case report from Ethiopian journal is another example for this.
Harlequin syndrome is very rare and unique in its presentation. Hence awareness of this case can help clinicians to evaluate and mangae such cases. Though it is idiopathic we have to rule out secondary causes before giving reassurance to the patients
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