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 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

Cyanosis Medical diagnosis

Cyanosis is the bluish or purplish discoloration of the skin or mucous membranes due to the tissues near the skin surface having low oxygen saturation. Based on Lundsgaard and Van Slyke's work, it is classically described as occurring if 5.0 g/dL of deoxyhemoglobin or greater is present. This was based on an estimate of capillary saturation based on a mean of arterial versus peripheral venous blood gas measurements. Since estimation of hypoxia is usually now based either on arterial blood gas measurement or pulse oximetry, this is probably an overestimate, with evidence that levels of 2.0 g/dL of deoxyhemoglobin may reliably produce cyanosis. Since, however, the presence of cyanosis is dependent upon there being an absolute quantity of deoxyhemoglobin, the bluish color is more readily apparent in those with high hemoglobin counts than it is with those with anemia. Also, the bluer the color, the more difficult it is to detect on deeply pigmented skin. When signs of cyanosis first appear, such as on the lips or fingers, intervention should be made within 3–5 minutes because a severe hypoxia or severe circulatory failure may have induced the cyanosis.

Ischemia Restriction in blood supply to tissues

Ischemia or ischaemia is a restriction in blood supply to tissues, 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 means local hypoxia in a given part of a body sometimes resulting from constriction. Ischemia comprises not only insufficiency of oxygen, but also reduced availability of nutrients and inadequate removal of metabolic wastes. Ischemia can be partial or total. The inadquate delivery of oxygenated blood to the organs must be resolved either by treating the cause of the inadequate delivery or reducing the oxygen demand of the system that needs it. For example, patients with myocardial ischemia have a decreased blood flow to the heart and are prescribed with medications that reduce chronotrophy and ionotrophy to meet the new level of blood delivery supplied by the stenosed so that it is adequate.

Chilblains Medical condition

Chilblains, also known as pernio and chill burns, are 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. It occurs most frequently when predisposed individuals, predominantly women, are exposed to cold and humidity. Ulcerated chilblains are referred to as kibes. Temperature-related chilblains can be prevented by keeping the feet and hands warm in cold weather and avoiding exposing these areas to extreme temperature changes. Once the diagnosis of chilblains is made, first-line treatment includes avoiding cold, damp environments and wearing gloves and warm socks.

Raynaud syndrome Medical condition in which spasm of arteries causes episodes of reduced blood flow

Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician, Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, 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, 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.

Blue baby syndrome Two situations that lead to cyanosis in infants

Blue baby syndrome can refer to a number of conditions that affect oxygen transportation in the blood, resulting in blueness of the skin in babies. Historically, the term "blue baby syndrome" has referred to babies with one of two conditions:

  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.
Erythromelalgia Medical condition

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 thrombocytosis, 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>

Harrison's Principles of Internal Medicine is an American textbook of internal medicine. First published in 1950, it is in its 20th edition and comes in two volumes. Although it is aimed at all members of the medical profession, it is mainly used by internists and junior doctors in this field, as well as medical students. It is widely regarded as one of the most authoritative books on internal medicine and has been described as the "most recognized book in all of medicine."

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.

CREST syndrome Medical condition

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.

Sinus bradycardia Sinus bradycardia is a sinus rhythm with a rate that is lower than normal

Sinus bradycardia is a sinus node dysfunction with a rate that is lower than normal. In humans, bradycardia is generally defined to be a rate of under 60 beats per minute. A normal heartbeat in human is usually at a rate of 60 to 100 beats per minute.

Sinus tachycardia Medical condition

Sinus tachycardia is an elevated sinus rhythm characterized by an increase in the rate of electrical impulses arising from the sinoatrial node. In adults, sinus tachycardia is defined as a heart rate greater than 100 beats/min (bpm). The normal resting heart rate is 60–100 bpm in an average male adult and 60-90 bpm in an average female adult. Normal heart rate varies with age, from infants having faster heart rates and the elderly having slower heart rates. Sinus tachycardia is a normal response to physical exercise, when the heart rate increases to meet the body's higher demand for energy and oxygen, but sinus tachycardia can also indicate a health problem. Thus, sinus tachycardia is a medical finding that can be either physiological or pathological.

Livedo reticularis Medical condition

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 through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration. This can be a secondary effect of a condition that increases a person's risk of forming blood clots, 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.

Iloprost

Iloprost is a medication used to treat pulmonary arterial hypertension (PAH), scleroderma, Raynaud's phenomenon and other diseases in which the blood vessels are constricted and blood cannot flow to the tissues. This damages the tissues and causes high blood pressure. There is ongoing research into using it as a frostbite treatment. Iloprost works by opening (dilating) the blood vessels to allow the blood to flow through again. It was developed by the pharmaceutical company Schering AG and is marketed by Bayer Schering Pharma AG in Europe and Actelion Pharmaceuticals in the USA.

Vascular disease Medical condition

Vascular disease is a class of diseases of the blood vessels – the arteries and veins of the circulatory system of the body. It is a subgroup of cardiovascular disease. Disorders in this vast network of blood vessels, can cause a range of health problems which can be severe or prove fatal.

Cutis marmorata telangiectatica congenita Medical condition

Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. The condition was first recognised 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.

Capillary leak syndrome is characterized by the escape of blood plasma through capillary walls, from the blood circulatory system to surrounding tissues, muscle compartments, organs or body cavities. It is a phenomenon most commonly witnessed in sepsis, and less frequently in autoimmune diseases, differentiation syndrome, engraftment syndrome, hemophagocytic lymphohistiocytosis, the ovarian hyperstimulation syndrome, viral hemorrhagic fevers, and snakebite and ricin poisoning. Pharmaceuticals, including the chemotherapy medications gemcitabine and tagraxofusp, as well as certain interleukins and monoclonal antibodies, can also cause capillary leaks. These conditions and factors are sources of secondary capillary leak syndrome.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which the chilling of an individual's blood plasma from the normal body temperature of 37 °C to the near-freezing temperature of 4 °C causes the reversible precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins. Returning this plasma to 37 °C resolubilizes the precipitate.

Superficial thrombophlebitis Medical condition

Superficial thrombophlebitis is a thrombosis and inflammation of superficial veins which presents as a painful induration with erythema, often in a linear or branching configuration forming cords.

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 (PE). 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.

Flammer syndrome is a described clinical entity comprising a complex of clinical features caused mainly by dysregulation of the blood supply. It was previously known as vascular dysregulation. It can manifest itself in many symptoms such as cold hands and feet and is often associated with low blood pressure. In certain cases it is associated with or predisposes for the development of diseases such as a normal tension glaucoma. Flammer syndrome is named after the Swiss ophthalmologist Josef Flammer.

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. ↑ "Acrocyanosis". Vascular Medicine: A Companion to Braunwald's Heart Disease: 600–603. 2013-01-01. doi:10.1016/B978-1-4377-2930-6.00049-5.
  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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