Acrocyanosis

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Acrocyanosis
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Dependent acrocyanosis in Postural orthostatic tachycardia syndrome patient
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Acrocyanosis is persistent blue or cyanotic discoloration of the extremities, most commonly occurring in the hands, although it also occurs in the feet and distal parts of the face. [1] [2] [3] [4] Although described over 100 years ago and not uncommon in practice, the nature of this phenomenon is still uncertain. [5] The very term "acrocyanosis" is often applied inappropriately in cases when blue discoloration of the hands, feet, or parts of the face is noted. [1] The principal (primary) form of acrocyanosis is that of a benign cosmetic condition, [2] sometimes caused by a relatively benign neurohormonal disorder. [3] Regardless of its cause, the benign form typically does not require medical treatment. A medical emergency would ensue if the extremities experience prolonged periods of exposure to the cold, particularly in children and patients with poor general health. [3] However, frostbite differs from acrocyanosis because pain (via thermal nociceptors) often accompanies the former condition, while the latter is very rarely associated with pain. There are also a number of other conditions that affect hands, feet, and parts of the face with associated skin color changes that need to be differentiated from acrocyanosis: Raynaud phenomenon, pernio, acrorygosis, erythromelalgia, and blue finger syndrome. The diagnosis may be challenging in some cases, especially when these syndromes co-exist. [1]

Contents

Acrocyanosis may be a sign of a more serious medical problem, such as connective tissue diseases and diseases associated with central cyanosis. Other causative conditions include infections, toxicities, antiphospholipid syndrome, cryoglobulinemia, neoplasms. In these cases, the observed cutaneous changes are known as "secondary acrocyanosis". They may have a less symmetric distribution and may be associated with pain and tissue loss. [1] [2]

Signs and symptoms

Acrocyanosis is characterized by peripheral cyanosis: persistent cyanosis of the hands, feet, knees, or face. [4] The extremities often are cold and clammy and may exhibit some swelling (especially in warmer weather). [1] [2] The palms and soles exhibit a wide range of sweating from moderately moist to profuse, but all peripheral pulses should have normal rate, rhythm, and quality. [3] [4] Exposure to cold temperatures worsens the cyanosis, while it often improves on warming. [2] Aside from the color changes, patients normally are asymptomatic and therefore there is usually no associated pain. The most common sign, discoloration, usually is what prompts patients to seek medical care. [3] [4]

Pathophysiology

The precise mechanism of acrocyanosis is not known. [6] The current line of thinking goes that vasospasms in the cutaneous arteries and arterioles produce cyanotic discoloration, while compensatory dilatation in the postcapillary venules causes sweating. Arteriovenous subpapillary plexus shunting also occurs. [1] [2] [4] Persistent vasoconstriction at the precapillary sphincter creates a local hypoxic environment, thus releasing adenosine into the capillary bed. [7] Vasospasms force adenosine to enter the capillary bed, where it vasodilates the postcapillary venules. [7] Such differences in vessel tone create a countercurrent exchange system that attempts to retain heat. Profuse sweating would then be caused by an overwhelmed countercurrent exchange system. In addition to adenosine, other hormones may contribute to acrocyanosis such as increase blood levels of serotonin. [8] This would seem to support case studies reporting acrocyanosis as an unusual side effect for pediatric patients taking tricyclic antidepressants, as these medications can inhibit the reuptake of serotonin and thus increase their blood concentrations. [9] Acrocyanosis has been reported in association with many other medications and substances. [1]

Diagnosis

Acrocyanosis is diagnosed clinically, based on a medical history and physical examination; laboratory studies or imaging studies are not necessary. The normal peripheral pulses rule out peripheral arterial occlusive disease, where arterial narrowing limits blood flow to the extremities. Pulse oximetry will show a normal oxygen saturation. Unlike the closely related Raynaud's phenomenon, cyanosis is continually persistent. In addition, there is usually no associated trophic skin changes, localized pain, or ulcerations. [2] [4] Capillaroscopy and other laboratory methods may be helpful but only complement clinical diagnosis in unclear cases, especially when connective tissue disorders may be present. [1]

Treatment

There is no standard medical or surgical treatment for acrocyanosis, and treatment, other than reassurance and avoidance of cold, is usually unnecessary. The patient is reassured that no serious illness is present. A sympathectomy would alleviate the cyanosis by disrupting the fibers of the sympathetic nervous system to the area. [3] However, such an extreme procedure would rarely be appropriate. Treatment with vasoactive drugs is not recommended but traditionally is mentioned as optional. However, there is little, if any, empirical evidence that vasoactive drugs (α-adrenergic blocking agents or calcium channel blockers) are effective. [1] [2]

Prognosis

While there is no cure for acrocyanosis, patients otherwise have excellent prognosis. [2] Unless acrocyanosis results from another condition (e.g. malignancy, antiphospholipid syndrome, atherosclerosis, acute ischemic limb, bacterial endocarditis), there is no associated increased risk of disease or death, and there are no known complications. Aside from the discoloration, there are no other symptoms: no pain, and no loss of function. Patients can expect to lead normal lives. In secondary acrocyanosis treatment of the primary condition defines outcomes. [1]

Epidemiology

Although there is no definitive reporting on its incidence, acrocyanosis shows prevalence in children and young adults than in patients thirty years of age or older. [3] Epidemiological data suggests that cold climate, outdoor occupation, and low body mass index are significant risk factors for developing acrocyanosis. [10] As expected, acrocyanosis would be more prevalent in women than in men due to differences in BMI. [4] However, the incidence rate of acrocyanosis often decreases with increasing age, regardless of regional climate. It completely resolves in many women after menopause implying significant hormonal influences. [1] [10]

Around 50% of patients with POTS experience acrocyanosis of their legs while standing still. [11] [12]

In the newborn

Acrocyanosis is common initially after delivery in the preterm and full term newborn. [13] Intervention is typically not required as it is seen as a normal finding. Acrocyanosis can also return in a newborn if a baby is cold, such as after a bath, and is considered normal as well. [14]

See also

Related Research Articles

<span class="mw-page-title-main">Cyanosis</span> Decreased oxygen in the blood

Cyanosis is the change of body tissue color to a bluish-purple hue, as a result of decrease in the amount of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Cyanosis is apparent usually in the body tissues covered with thin skin, including the mucous membranes, lips, nail beds, and ear lobes. Some medications may cause discoloration such as medications containing amiodarone or silver. Furthermore, mongolian spots, large birthmarks, and the consumption of food products with blue or purple dyes can also result in the bluish skin tissue discoloration and may be mistaken for cyanosis. Appropriate physical examination and history taking is a crucial part to diagnose cyanosis. Management of cyanosis involves treating the main cause, as cyanosis isn’t a disease, it is a symptom.

<span class="mw-page-title-main">Ischemia</span> Restriction in blood supply to tissues

Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism. Ischemia is generally caused by problems with blood vessels, with resultant damage to or dysfunction of tissue i.e. hypoxia and microvascular dysfunction. It also implies local hypoxia in a part of a body resulting from constriction.

<span class="mw-page-title-main">Chilblains</span> Inflammation due to damage of skin capillaries when blood perfuses into nearby tissue

Chilblains, also known as pernio, is a medical condition in which damage occurs to capillary beds in the skin, most often in the hands or feet, when blood perfuses into the nearby tissue, resulting in redness, itching, inflammation, and possibly blisters.

<span class="mw-page-title-main">Raynaud syndrome</span> Medical condition in which spasm of arteries causes episodes of reduced blood flow

Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which the spasm of small arteries causes episodes of reduced blood flow to end arterioles. Typically the fingers, and, less commonly, the toes, are involved. Rarely, the nose, ears, nipples, or lips are affected. The episodes classically result in the affected part turning white and then blue. Often, numbness or pain occurs. As blood flow returns, the area turns red and burns. The episodes typically last minutes but can last several hours. The condition is named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862.

Microvascular angina (MVA), previously known as cardiac syndrome X, also known as coronary microvascular dysfunction(CMD) or microvascular coronary disease is a type of angina (chest pain) with signs associated with decreased blood flow to heart tissue but with normal coronary arteries.

<span class="mw-page-title-main">Blue baby syndrome</span> Two situations that lead to cyanosis in infants

Blue baby syndrome can refer to conditions that cause cyanosis, or blueness of the skin, in babies as a result of low oxygen levels in the blood. This term has traditionally been applied to cyanosis as a result of:.

  1. Cyanotic heart disease, which is a category of congenital heart defect that results in low levels of oxygen in the blood. This can be caused by either reduced blood flow to the lungs or mixing of oxygenated and deoxygenated blood.
  2. Methemoglobinemia, which is a disease defined by high levels of methemoglobin in the blood. Increased levels of methemoglobin prevent oxygen from being released into the tissues and result in hypoxemia.
<span class="mw-page-title-main">Erythromelalgia</span> Inflammation due to periodic blood vessel blockage

Erythromelalgia or Mitchell's disease is a rare vascular peripheral pain disorder in which blood vessels, usually in the lower extremities or hands, are episodically blocked, then become hyperemic and inflamed. There is severe burning pain and skin redness. The attacks are periodic and are commonly triggered by heat, pressure, mild activity, exertion, insomnia or stress. Erythromelalgia may occur either as a primary or secondary disorder. Secondary erythromelalgia can result from small fiber peripheral neuropathy of any cause, polycythemia vera, essential thrombocythemia, hypercholesterolemia, mushroom or mercury poisoning, and some autoimmune disorders. Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel α-subunit gene SCN9A.

<i>Harrisons Principles of Internal Medicine</i> American internal medicine textbook

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Intermittent claudication, also known as vascular claudication, is a symptom that describes muscle pain on mild exertion, classically in the calf muscle, which occurs during exercise, such as walking, and is relieved by a short period of rest. It is classically associated with early-stage peripheral artery disease, and can progress to critical limb ischemia unless treated or risk factors are modified and maintained.

<span class="mw-page-title-main">CREST syndrome</span> Connective tissue disorder

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

<span class="mw-page-title-main">Sinus bradycardia</span> Sinus rhythm with a rate that is lower than normal

Sinus bradycardia is a sinus rhythm with a reduced rate of electrical discharge from the sinoatrial node, resulting in a bradycardia, a heart rate that is lower than the normal range.

<span class="mw-page-title-main">Sinus tachycardia</span> Sinus rhythm with a rate that is higher than normal

Sinus tachycardia is a sinus rhythm of the heart, with an increased rate of electrical discharge from the sinoatrial node, resulting in a tachycardia, a heart rate that is higher than the upper limit of normal.

<span class="mw-page-title-main">Livedo reticularis</span> Purplish discoloration of the skin due to reduced blood flow

Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by reduction in blood flow (ischemia) through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration (cyanosis). This can be a secondary effect of a condition that increases a person's risk of forming blood clots (thrombosis), including a wide array of pathological and nonpathological conditions. Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.

<span class="mw-page-title-main">Vascular disease</span> Medical condition

Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.

<span class="mw-page-title-main">Cutis marmorata telangiectatica congenita</span> Medical condition

Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. The condition was first recognized and described in 1922 by Cato van Lohuizen, a Dutch pediatrician whose name was later adopted in the other common name used to describe the condition – Van Lohuizen syndrome. CMTC is also used synonymously with congenital generalized phlebectasia, nevus vascularis reticularis, congenital phlebectasia, livedo telangiectatica, congenital livedo reticularis and Van Lohuizen syndrome.

Blue toe syndrome is a situation that may reflect atherothrombotic microembolism, causing transient focal ischaemia, a temporary blockage of blood flow to the brain or spinal cord, occasionally with minor apparent tissue loss, but without diffuse forefoot ischemia. The development of blue or violaceous toes can also occur with trauma, cold-induced injury, disorders producing generalized cyanosis, decreased arterial flow, impaired venous outflow, and abnormal circulating blood. The terms "blue toe syndrome", "grey toe syndrome" and "purple toe syndrome" are sometimes used interchangeably.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which a person's blood plasma is allowed to cool substantially, causing the (reversible) precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins.

<span class="mw-page-title-main">Cold injury</span> Medical condition

Cold injury is damage to the body from cold exposure, including hypothermia and several skin injuries. Cold-related skin injuries are categorized into freezing and nonfreezing cold injuries. Freezing cold injuries involve tissue damage when exposed to temperatures below freezing. Nonfreezing cold injuries involve tissue damage when exposed to temperatures often between 0-15 degrees Celsius for extended periods of time. While these injuries have disproportionally affected military members, recreational winter activities have also increased the risk and incidence within civilian populations. Additional risk factors include homelessness, inadequate or wet clothing, alcohol abuse or tobacco abuse, and pre-existing medical conditions that impair blood flow.

Blood clots are a relatively common occurrence in the general population and are seen in approximately 1-2% of the population by age 60. Typically, blood clots develop in the deep veins of the lower extremities, deep vein thrombosis (DVT) or as a blood clot in the lung, pulmonary embolism. A very small number of people who develop blood clots have a more serious and often life-threatening condition, known as thrombotic storm (TS). TS is characterized by the development of more than one blood clot in a short period of time. These clots often occur in multiple and sometimes unusual locations in the body and are often difficult to treat. TS may be associated with an existing condition or situation that predisposes a person to blood clots, such as injury, infection, or pregnancy. In many cases, a risk assessment will identify interventions that will prevent the formation of blood clots.

Non-freezing cold injuries (NFCI) is a class of tissue damage caused by sustained exposure to low temperature without actual freezing. There are several forms of NFCI, and the common names may refer to the circumstances in which they commonly occur or were first described, such as trench foot, which was named after its association with trench warfare. NFCI is caused by microvascular endothelial damage, stasis and vascular occlusion and is characterised by peripheral neuropathy. NFCI generally affects the hands or feet during exposure to temperatures just above freezing, often wet, and is typically found in soldiers.

References

  1. 1 2 3 4 5 6 7 8 9 10 11 Kurklinsky AK, Miller VM, Rooke TW. "Acrocyanosis: The Flying Dutchman." Vascular Medicine 2011 Aug;16(4):288-301
  2. 1 2 3 4 5 6 7 8 9 Olin, J.W. (2004). Other peripheral arterial diseases. In L. Goldman & D. Ausiello (Eds.), Cecil Textbook of Medicine, 22nd Edition. (Vol 1, pp. 475). Philadelphia: WB Saunders ISBN   0-7216-9653-8
  3. 1 2 3 4 5 6 7 Creager, M.A. & Dzau, V.J. (2005). Vascular diseases of the extremities. In D.L. Kasper, A.S. Fauci, D.L. Longo, E. Braunwald, S.L. Hauser, & J.L Jameson (Eds.), Harrison's Prins of Internal Medicine (16th ed., pp. 1490). New York: McGraw-Hill ISBN   0-07-140235-7
  4. 1 2 3 4 5 6 7 (2006). Peripheral vascular disorders. In R.S. Porter, T.V. Jones, & M.H. Beer (Eds.), Merck Manual of Diagnosis and Therapy (18th ed., ch. 212). New York: Wiley, John & Sons ISBN   0-911910-18-2
  5. ↑ Coffman, Jay D. (2006), "Acrocyanosis", Vascular Medicine, Elsevier, pp. 707–710, doi:10.1016/b978-0-7216-0284-4.50055-5, ISBN   978-0-7216-0284-4 , retrieved 2021-07-30
  6. ↑ Chadachan, Veerendra; Eberhardt, Robert T. (2013-01-01). "Acrocyanosis". Vascular Medicine: A Companion to Braunwald's Heart Disease: 600–603. doi:10.1016/B978-1-4377-2930-6.00049-5. ISBN   9781437729306.
  7. 1 2 Guyton, A.C. & Hall, J.E. (2006) Textbook of Medical Physiology (11th ed.) Philadelphia: Elsevier Saunder ISBN   0-7216-0240-1
  8. ↑ Carpenter, PK; Morris, D (1990). "Association of acrocyanosis with Asperger's syndrome". Journal of Mental Deficiency Research. 34 (1): 87–90. doi:10.1111/j.1365-2788.1990.tb01519.x. PMID   2325122.
  9. ↑ Karakara, I.; Aydoğan, M.; Coşkun, A.; Gökalp, A.S. (2003). "Acrocyanosis as a side effect of tricyclic antidepressants: A case report". The Turkish Journal of Pediatrics. 45 (2): 155–57. PMID   12921305.
  10. 1 2 Carpentier, P.H. (1998). "Definition and Epidemiology of Vascular Acrosyndromes". Rev Prat. 48 (15): 1641–6. PMID   9814064.
  11. ↑ Abou-Diab, Jane; Moubayed, Dina; Taddeo, Danielle; Jamoulle, Olivier; Stheneur, Chantal (2018). "Acrocyanosis Presentation in Postural Orthostatic Tachycardia Syndrome". International Journal of Clinical Pediatrics. 7 (1–2): 13–16. doi: 10.14740/ijcp293w . ISSN   1927-1255.
  12. ↑ Raj, Satish R. (2006-04-01). "The Postural Tachycardia Syndrome (POTS): pathophysiology, diagnosis & management". Indian Pacing and Electrophysiology Journal. 6 (2): 84–99. ISSN   0972-6292. PMC   1501099 . PMID   16943900.
  13. ↑ Engle, W.A. & Boyle, D.W. (2005). Delivery room management and transitional care. In L.M. Osborn, T.G. DeWitt, L.R. First, & J.A. Zenel (Eds.), Pediatrics (pp. 1250-61). Philadelphia: Elsevier Mosby ISBN   0-323-01199-3
  14. ↑ "Skin Color Changes in the Newborn". Saint Luke's Health System. Retrieved 2020-04-08.
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