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.
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References
Grubb BP, Kanjwal Y, Kosinski DJ. The postural tachycardia syndrome: a concise guide to diagnosis and management. J Cardiovasc Electrophysiol. 2006;17:108–12.
Freeman R, Wieling W, Axelrod FB, Benditt DG, Benarroch E, Biaggioni I, et al. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Clin Auton Res. 2011;21:69–72.
Benarroch EE. Postural tachycardia syndrome: a heterogeneous and multifactorial disorder. Mayo Clin Proc. 2012;87(12):1214–25.
Jacob G, Costa F, Shannon JR. The neuropathic postural tachycardia syndrome. N Engl J Med. 2000;343(14):1008–14.
Jordan J, Shannon JR, Diedrich A, Black BK, Robertson D. Increased sympathetic activation in idiopathic orthostatic intolerance: role of systemic adrenoreceptor sensitivity. Hypertension. 2002;39:173–8.
Raj SR, Robertson D. Blood volume perturbations in the postural tachycardia syndrome. Am J Med Sci. 2007;334(1):57–60.
Joyner MJ, Masuki S. POTS versus deconditioning: the same or different? Clin Auton Res. 2008;18(6):300–7.
Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R. Postural tachycardia syndrome—current experience and concepts. Nat Rev Neurol. 2011;8(1):22–34.
Low PA, Sandroni P, Joyner M, Shen WK. Postural tachycardia syndrome (POTS). J Cardiovasc Electrophysiol. 2009;20(3):352–8.
Ojha A, Chelimsky TC, Chelimsky G. Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome. J Pediatr. 2011;158(1):20–3.
Landero J. Postural orthostatic tachycardia syndrome: a dermatologic perspective and successful treatment with losartan. J Clin Aesthet Dermatol. 2014;7(8):41–7.
Kanjwal K, Saeed B, Karabin B, Kanjwal Y, Grubb BP. Comparative clinical profile of postural orthostatic tachycardia patients with and without joint hypermobility syndrome. Indian Pacing Electrophysiol J. 2010;10(4):173–8.
Wallman D, Weinberg J, Hohler AD. Ehlers-Danlos syndrome and Postural tachycardia syncrome: a relationship study. J Neurol Sci. 2014;340(1–2):99–102.
Marshall JS, Jawdat DM. Mast cells in innate immunity. J Allergy Clin Immunol. 2004;114(1):21.
Niimi N, Francis DM, Kermani F, O’Donnell BF, Hide M, Kobza-Black A, et al. Dermal mast cell activation by autoantibodies against the high affinity IgE receptor in chronic urticaria. J Invest Dermatol. 1996;106:1001–6.
Sabroe RA. Acute urticaria. Immunol Allergy Clin N Am. 2014;34(1):11–21.
Bryce PJ, Mathias CB, Harrison KL, Watanabe T, Geha RS, Oettgen HC. The H1 histamine receptor regulates allergic lung responses. J Clin Invest. 2006;116(6):1624.
Wilkin JK. Oral thermal-induced flushing in erythemato-telangiectatic rosacea. J Invest Dermatol. 1981;76:15–8.
Ekman P, Levenson RW, Friesen WV. Autonomic nervous system activity distinguishes among emotions. Science. 1983;221:1208–10.
Izikson L, English JC, Zirwas MJ. The flushing patient: differential diagnosis, work up, and treatment. J Am Acad Dermatol. 2006;55:193–208.
Altrichter S, Wosny K, Maurer M. Successful treatment of cholinergic urticaria with methantheliniumbromide. J Dermatol. 2015;42:422–4.
Roberts LJ, Oates JA. Biochemical diagnosis of systemic mast cell disorders. J Invest Dermatol. 1991;96:19S–24S.
Frieri M, Patel R, Celestin J. Mast cell activation syndrome: a review. Curr Allergy Asthma Rep. 2012;13:27–32.
Shibao C, Arzubiaga C, Roberts LJ 2nd, Raj S, Black B, Harris P, et al. Hyperadrenergic postural tachycardia syndrome in mast cell activation disorders. Hypertension. 2005;45:385–90.
Hogan SR, Mandrell J, Eilers D. Adrenergic urticaria: review of the literature and proposed mechanism. J Am Acad Dermatol. 2014;70:763–6.
Shelley WB, Shelley ED. Adrenergic urticaria: a new form of stress-induced hives. Lancet. 1985;8643:1031–3.
Haustein UF. Adrenergic urticaria and adrenergic pruritus. Acta Derm Venereol. 1990;70:82–4.
Butendieck RR, Murray PM. Raynaud disease. J Hand Surg Am. 2014;39(1):121–4.
Herrick AL. Pathogenesis of Raynaud’s phenomenon. Rheumatol (Oxf). 2005;44:587–96.
Zamora MR, O’Brien RF, Rutherford RB, Weil JV. Serum endothelin-1 concentrations and cold provocation in primary Raynaud’s phenomenon. Lancet. 1990;336:1144–7.
Stewart JM, Ocon AJ, Clarke D, Taneja I, Medow MS. Defects in cutaneous angiotensin-II converting enzyme 2 and angiotensin-(1–7) production in postural tachycardia syndrome. Hypertension. 2009;53:767–74.
Ferrario CM, Trask AJ, Jessup JA. Advances in biochemical and functional roles of angiotensin-converting enzyme 2 and angiotensin-(1–7) in regulation of cardiovascular function. Am J Physiol Heart Circ Physiol. 2005;289:H2281–90.
Stewart JM, Taneja I, Medow MS. Reduced body mass index is associated with increased angiotensin-II in young women with postural tachycardia syndrome. Clin Sci (Lond). 2007;113:449–57.
Stewart JM, Taneja I, Glover J, Medow MS. Angiotensin II type 1 receptor blockade corrects cutaneous nitric oxide deficit in postural tachycardia syndrome. Am J Physiol Heart Circ Physiol. 2008;294:H466–73.
Gao L, Wang W, Li YL, Schultz HD, Liu D, Cornish KG, et al. Sympathoexcitation by central ANG II: roles for AT1 receptor upregulation and NAD(P)H oxidase in RVLM. Am J Physiol Heart Circ Physiol. 2005;288:H2271–9.
Harrison DG, Cai H, Landmesser U, Griendling KK. Interactions of angiotensin II with NAD(P)H oxidase, oxidant stress and cardiovascular disease. J Renin Angiotensin Aldosterone Syst. 2003;4:51–61.
Fleischer AB, Resnick SD. Livedo reticularis. Dermatol Clin. 1990;8(2):347–54.
Gibbs MB, English JC 3rd, Zirwas MJ. Livedo reticularis: an update. J Am Acad Dermatol. 2005;52(6):1009–19.
Naldi L, Locati F, Marchesi L, Cortelazzo S, Finazzi G, Galli M, et al. Cutaneous manifestations associated with antiphospholipid antibodies in patients with suspected primary antiphospholipid syndrome: a case–control study. Ann Rheum Dis. 1993;52:219–22.
Kester S, McCarty DL, McCarty GA. The antiphospholipid antibody syndrome in the emergency department setting—livedo reticularis and recurrent venous thrombosis. Ann Emerg Med. 1992;21:207–11.
Speight EL, Lawrence CM. Reticulate purpura, cryoglobulinaemia and livedo reticularis. Br J Dermatol. 1993;129:319–23.
Abrahamian LM, Berke A, Van Voorhees AS. Type 1 diabetes mellitus associated with livedo reticularis: case report and review of the literature. Pediatr Dermatol. 1991;8:46–50.
Sevastos NS, Rafailidis PI, Katoulis AC, Kolokotronis KG, Stavrianeas NG. Livedo reticularis associated with pernicious anemia. Clin Exp Dermatol. 2003;28:447–59.
Chen L, Wang L, Sun J, Qin J, Tang C, Jin H, et al. Midodrine hydrochloride is effective in the treatment of children with postural orthostatic tachycardia syndrome. Circ J. 2011;75(4):927–31.
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The authors, Hao Huang and Anna DePold Hohler, have no conflicts of interest to declare.
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Huang, H., Hohler, A.D. The Dermatological Manifestations of Postural Tachycardia Syndrome: A Review with Illustrated Cases. Am J Clin Dermatol 16, 425–430 (2015). https://doi.org/10.1007/s40257-015-0144-6
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DOI: https://doi.org/10.1007/s40257-015-0144-6