Successful treatment of idiopathic Harlequin Syndrome with oxybutynin and propranolol

• Published in: Australasian journal of dermatology 2021-11, Vol.62 (4), p.504-505
• Main Authors: Naharro‐Fernández, Cristina, Quintana‐Sancho, Adrián, López‐Sundh, Ana Elizabet, Reguero‐del Cura, Leandra, Gónzalez‐López, Marcos A.
• Format: Article
• Language: English
• Subjects: Autonomic Nervous System Diseases - diagnosis; Autonomic Nervous System Diseases - drug therapy; Erythema; Female; Flushing - diagnosis; Flushing - drug therapy; harlequin syndrome; Humans; hyperhidrosis; Hypohidrosis - diagnosis; Hypohidrosis - drug therapy; Mandelic Acids - therapeutic use; Middle Aged; oxibutinine; Parasympatholytics - therapeutic use; Patients; propanolol; Propranolol; Propranolol - therapeutic use; Quality of life; Sweating; Sympathetic nervous system; Vasodilator Agents - therapeutic use
• ISSN: 0004-8380; 1440-0960
• DOI: 10.1111/ajd.13665

Harlequin syndrome (HS) is a rare entity derived from the dysfunction of the sympathetic nervous system. It is characterised by unilateral facial flushing and sweating induced by exercise, heat and emotion. Most cases are primary with an unknown pathogenic mechanism. In these cases, the prognosis is favourable. Medical or surgical treatments are not usually required for idiopathic HS. However, symptomatic treatment may be indicated when symptoms affect the quality of life of patients. We present the case of a patient with idiopathic HS successfully treated with oxybutynin and propranolol. In this patient, a marked improvement in both hyperhidrosis and facial erythema was noted with this combined therapy. We consider it of interest to highlight the response of our patient to the treatment employed, which may be advantageous in future cases of this rare disorder.