Dermatologic therapy
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Dermatologic therapy · Jul 2014
Case ReportsIntractable vascular autonomic dysregulation (Harlequin phenomenon) in two brothers: another indication for propranolol?
Vascular autonomic dysregulation, in the most extreme presentation known as Harlequin phenomenon, is a rare condition. It manifests as a sudden and brief paroxystic change in skin color, resulting in two different colors on the body. It is supposed that this condition occurs due to a vasomotor instability. ⋯ These patients were treated with propranolol, a nonselective beta-blocker, resulting in improvement of symptoms: only occasional attacks were seen. Beta-blockers act on β1 -adrenoceptors in the heart, thereby preventing the positive chronotropic and inotropic effects mediated by these receptors. We hypothesize that propranolol, which is very lipophilic and therefore also acts on β-receptors of the central nervous system, acts on the sympathetic system.