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D. Bessis, V. Pallure, É. Jeziorski, BASCULE syndrome, orthostatic cyanosis and postural orthostatic tachycardia syndrome: time for decanting old wine?, British Journal of Dermatology, Volume 175, Issue 5, 1 November 2016, Pages 1110–1111, https://doi.org/10.1111/bjd.14918
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Funding sources: None.
Conflicts of interest: None declared.
Dear Editor, We thank Dr McGibbon for his comments1 on BASCULE syndrome (Bier anaemic spots associated with cyanosis and urticaria‐like eruption).2
The author suggests that this syndrome is essentially a redescription of orthostatic acrocyanosis associated with chronic orthostatic intolerance and postural tachycardia syndrome (POTS), as previously reported by Stewart.3 We would like to address this assertion.
Although we agree that our description of BASCULE syndrome in adult patients is close to the picture that Stewart labelled ‘acrocyanosis in POTS’, the descriptive terms of orthostatic acrocyanosis or acrocyanosis appear unclear and inappropriate in our case. Indeed, all forms of cyanosis of the extremities are usually worsened by orthostatism, and the cyanosis associated with POTS is not confined to the hand and foot, but has a more widespread extension to the affected limbs. Moreover, the term acrocyanosis does not cover all of the cutaneous semiology observed in BASCULE syndrome. The association of Bier spots and an urticaria‐like eruption composed of red‐orange macules, sometimes centred by red pinpoint (1‐mm) macules, appears to be a discriminating clinical sign that is not seen in primary or secondary acrocyanosis. Likewise, BASCULE syndrome is clearly distinguishable from idiopathic Bier spots or Bier spots secondary to mixed cryoglobulinaemia, for example. In our opinion, the description of BASCULE as a distinct syndrome therefore seems appropriate.
