PA11: Bier anaemic spots, cyanosis and urticaria-like eruption (BASCULE) syndrome: a case series of four patients

R. Fisher and T. McPherson

Oxford University Hospitals NHS Foundation Trust, Oxford, UK

Bier anaemic spots, cyanosis and urticaria-like eruption (BASCULE) syndrome is a recently described phenomenon, first reported in the literature in 2016 [Bessis D, Jeeziorrski E, Rigau V et al. Bier anaemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome: a new entity? Br J Dermatol 2016; 175: 218–20]. Patients present with characteristic skin changes of white asymptomatic macules followed by dusky blue/grey skin changes and then itchy or painful urticated plaques on lower limbs, which appear after a few minutes of standing. Symptoms resolve with sitting or moving the affected limb(s). BASCULE syndrome has a female sex predominance and has been reported in patients between the ages of 3 months and 20 years. There are no long-term studies, but, as it not reported in adults, it is thought that it may resolve over time. Although the pathophysiology is not well understood, it is thought to represent an exaggerated normal vasomotor response. BASCULE syndrome has been reported to be associated with orthostatic intolerance and patients can experience presyncopal and syncopal episodes (Bessis et al.). Patients should undergo routine assessment to investigate for postural orthostatic tachycardia syndrome (POTS) or postural symptoms without tachycardia if there is any concern. BASCULE syndrome is treated initially by trying to minimize prolonged periods of standing, the wearing of compression stockings and trialling antihistamines. If orthostatic intolerance is associated, medications such as midodrine and ivabradine are considered. We present a case series of four patients (three females, one male; age range 11–15 years). All four patients presented with the classical skin changes of BASCULE syndrome after a period (of varying lengths) of standing that was reproducible in the clinic. Two patients were found incidentally when attending the paediatric clinic for unrelated health conditions. Two patients reported syncopal symptoms on standing, but examination did not reveal any significant abnormality or a definite diagnosis of POTS. Both of these patients still report ongoing orthostatic symptoms after 3 and 1 years, respectively, despite conservative treatment measures. There were no other significant health conditions. Our case series supports BASCULE syndrome as a benign condition that represents a vasomotor response. Although syncopal symptoms occur, most patients do not meet the criteria for POTS or require further intervention. Patients can be managed by good education and sensible conservative measures. Longer-term studies are required to inform prognosis.