Harlequin Syndrome: Unraveling the Complexities of Etiology, Clinical Presentation, and Management: A Comprehensive Review
Harlequin Syndrome (HS) is a rare autonomic disorder characterized by distinctly demarcated facial discoloration and increased sweating on one side of the face. While idiopathic cases are common, secondary etiologies are increasingly recognized, including iatrogenic causes such as surgical procedures and anesthesia. This review provides an overview of HS, including its etiology, clinical presentation, and management strategies. The etiology of HS is multifactorial, with idiopathic cases comprising a majority. Secondary causes include compression of sympathetic trunks, autoimmune diseases, and iatrogenic factors. The pathophysiology involves disruption of sympathetic flow to the affected side of the face, resulting in compensatory flushing and sweating on the opposite side. Clinical presentation typically includes unilateral facial flushing and sweating, often triggered by physical activity or emotions. HS may coexist with other neurological syndromes, posing diagnostic challenges. Management options range from reassurance for benign cases to surgical interventions or botulinum toxin injections for symptomatic relief. Despite its benign nature, HS can have a significant psychological impact on patients. Increased awareness among healthcare professionals and society is crucial for proper diagnosis and management, ensuring individuals with HS receive the necessary support and care.
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