Type of publication:
*Shaw, Roberta; *Oliwieckim Simone
British Journal of Dermatology. Conference: 102nd Annual Meeting of the British Association of Dermatologists. Glasgow United Kingdom. 187(Supplement 1) (pp 206), 2022. Date of Publication: July 2022.
A 15-year-old boy presented to the paediatric dermatology clinic with an unusual bilateral intermittent rash to his lower legs, which he had noticed for many years. The patient demonstrated multiple irregular white macules and urticated papules on a background of cyanosis of the legs when standing for prolonged periods, such as in the shower, and which was relieved by sitting. The mother believed that the rash began after a flu-like illness, and aside from this he was usually fit and well, with no significant past medical history, family history or regular medications. The patient had a full blood screen, including coagulation screen, which were normal. The patient described an intermittent lightheaded sensation on standing. Therefore, while in clinic the patient had a lying and standing blood pressure, which demonstrated a significant systolic drop from 137 mmHg lying to 109 mmHg standing, while his heart rate remained stable. The clinical findings suggested a diagnosis of BASCULE syndrome. BASCULE syndrome is a benign vasomotor dermatosis first described in 2016. The clinical findings described include Bier anaemic spots, cyanosis and urticaria-like eruption. The pathogenic mechanism remains unknown but is thought to be associated with transient tissue hypoxia through arteriolar vasoconstriction causing a paradoxical urticated rash that is worse on standing. Although BASCULE syndrome has been described as a separate entity, there have been links to POTS (postural orthostatic tachycardia syndrome) and orthostatic acrocyanosis, and so these diagnoses should be considered when investigating a patient with similar features.