Periodic facial erythema in a patient with postural orthostatic tachycardia syndrome

Abstract

A 27-year-old woman had been attending our Dermatology department for the ongoing management of longstanding periodical facial erythema. These episodes were associated with a burning sensation and, were typically brought on by heat or exercise and were relieved only with cooling measures such as sitting in front of a fan. The patient had noted that the flares were becoming more frequent (occurring almost daily) and more severe. Initially, the episodes were unilateral but over time, they became bilateral in distribution.

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

“…Mast cell activation and chemical sensitivities were studied for their association with postural orthostatic tachycardia syndrome (POTS) and Ehler Danlos syndrome (EDS) [ 18 ]. Facial periodic erythema has also been reported [ 19 ]. Connective tissue disorders, especially Ehler Danlos syndrome hypermobility type, have a high concurrence rate with POTS as found by Cohen and Markham [ 20 ].…”

Current pain management strategies for patients with erythromelalgia: a critical review

“…There are isolated reports of facial, auricular and genital involvement 8–10. EM can present at any age with onset commonly reported in the first decade of life, although it is most often diagnosed in the fifth and sixth decades 11.…”

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 Transient decreased levels of nitric oxide and increased angiotensin-II result in intermittent vasoconstriction and hypoxia. 1,2 Our patient manifested many of these symptoms and was diagnosed with POTS at Children's Hospital of Philadelphia's Acquired Autonomic Dysfunction Program.…”

“…Postural orthostatic tachycardia syndrome (POTS) is a poorly understood condition defined by autonomic dysfunction with abrupt tachycardia in the absence of orthostatic hypotension with standing or upright tilt. 1,2 Patients can exhibit a myriad of symptoms, including fatigue, exercise intolerance, presyncope, syncope, migraines, "brain fog," anxiety, gastrointestinal upset, or sleep disturbance.…”