The Dermatological Manifestations of Postural Tachycardia Syndrome: A Review with Illustrated Cases

Abstract

Postural tachycardia syndrome (POTS) is a syndrome of excessive tachycardia with orthostatic challenge, and relief of such symptoms with recumbence. There are several proposed subtypes of the syndrome, each with unique pathophysiology. Numerous symptoms such as excessive tachycardia, lightheadedness, blurry vision, weakness, fatigue, palpitations, chest pain, and tremulousness are associated with orthostatic intolerance. Other co-morbid conditions associated with POTS are not clearly attributable to orthostatic intolerance. These include chronic headache, fibromyalgia, functional gastrointestinal or bladder disorders, cognitive impairment, and sleep disturbances. Dermatological manifestations of POTS are also common and wide ranging, from livedo reticularis to Raynaud's phenomenon, from cutaneous flushing to erythromelalgia. Here, we provide three illustrative cases of POTS with dermatological manifestations. We discuss the potential pathophysiology underlying such dermatological manifestations, and how such mechanisms could in turn help guide development of management.

Postural Orthostatic Tachycardia Syndrome and Its Unusual Presenting Complaints in Women: A Literature Minireview

“…A wide array of dermatologic manifestations are found in association with POTS, which may mislead diagnosis. According to Huang et al, dermatologic manifestations range from livedo reticularis to Raynaud's phenomenon, from cutaneous flushing to erythromelalgia [ 17 ]. Mast cell activation and chemical sensitivities were studied for their association with postural orthostatic tachycardia syndrome (POTS) and Ehler Danlos syndrome (EDS) [ 18 ].…”

A 15‐year‐old girl with transient patches of erythema

“…Some of the more common are livedo reticularis, Raynaud phenomenon, dysesthesias, acrocyanosis, koilonychia, evanescent hyperemia, and erythromelalgia. 1,3 The most characteristic skin finding is evanescent hyperemia as observed in our patient, which is likely under-recognized. The newly described BASCULE (Bier anemic spots, cyanosis, and urticaria-like eruption) syndrome must be considered in patients presenting with skin changes and autonomic dysfunction.…”

“…2 The evidence for efficacy of any medication for cutaneous manifestations is sparse and at anecdotal case report level. 2,3 For evanescent hyperemia, topical antihistaminergic drugs and vasomodulators can be considered. In one case, losartan was shown to produce a marked decrease in flares of cutaneous disease.…”

Periodic facial erythema in a patient with postural orthostatic tachycardia syndrome

“…She had been diagnosed 3 years earlier by a consultant cardiologist when an active stand test showed an increased heart rate from 60 bpm when supine to a maximum of 131 bpm after 10 min standing upright, with only a slight fall in systolic blood pressure of 10 mmHg. Several dermatological manifestations of POTS appear to be common, including livedo reticularis, acrocyanosis, Raynaud phenomenon, flushing and evanescent hyperaemia . We believe our patient's painful facial and acral flushing episodes are likely to be a variation of erythromelalgia secondary to POTS.…”