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

Hao Huang; Anna DePold Hohler

Disclosures

Am J Clin Dermatol. 2015;16(5):425-430. 

In This Article

Abstract and Introduction

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.

Introduction

Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with postural change from supine to standing or with head-up tilt, and relief of such symptoms with recumbence.[1] The most recent consensus statement defines POTS as a sustained heart rate increment of 30 beats/min (adults) or 40 beats/min (age 12–19 years) within 10 min of standing or head-up tilt in the absence of orthostatic hypotension.[2]

While the pathophysiology of POTS as a syndrome of orthostatic intolerance is likely heterogeneous, several subtypes of the syndrome and their proposed potentially interacting mechanisms have been described ( Table 1 ).[3–7] Symptoms of orthostatic intolerance that are evidenced in POTS include those of cerebral hypoperfusion and reflex sympathetic activation that are exacerbated by heat, exertion, heavy meals, prolonged recumbency, menses, and vasoactive medications.[8] Nevertheless, many patients experience chronic and potentially disabling symptoms that cannot be mechanistically explained by postural tachycardia. These include chronic fatigue, exercise intolerance, and sleep disturbances.[3,9,10]

Included in the non-orthostatic symptoms of POTS are various dermatological and soft tissue conditions such as Raynaud's syndrome,[10] evanescent hyperemia, acrocyanosis, livedo reticularis (LR),[11] and Ehlers-Danlos syndrome.[12,13] Here, we identified three patients with dermatological manifestations of POTS, and we have included photographs and a discussion of possible mechanisms.

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