Acrocyanosis is persistent blue or
cyanotic
Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...
discoloration of the extremities, most commonly occurring in the hands, although it also occurs in the feet and distal parts of face.
[Kurklinsky AK, Miller VM, Rooke TW. "Acrocyanosis: The Flying Dutchman." ''Vascular Medicine'' 2011 Aug;16(4):288-301][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 ][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 ][(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 ] Although described over 100 years ago and not uncommon in practice, the nature of this phenomenon is still uncertain. The very term "acrocyanosis" is often applied inappropriately in cases when blue discoloration of the hands, feet, or parts of the face is noted.
The principal (primary) form of acrocyanosis is that of a
benign
Malignancy () is the tendency of a medical condition to become progressively worse.
Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...
cosmetic condition,
sometimes caused by a relatively benign
neurohormonal disorder.
Regardless of its cause, the benign form typically does not require medical treatment. A
medical emergency
A medical emergency is an acute injury or illness that poses an immediate risk to a person's life or long-term health, sometimes referred to as a situation risking "life or limb". These emergencies may require assistance from another, qualified p ...
would ensue if the extremities experience prolonged periods of exposure to the cold, particularly in children and patients with poor general health.
However,
frostbite
Frostbite is a skin injury that occurs when exposed to extreme low temperatures, causing the freezing of the skin or other tissues, commonly affecting the fingers, toes, nose, ears, cheeks and chin areas. Most often, frostbite occurs in the han ...
differs from acrocyanosis because pain (via thermal
nociceptor
A nociceptor ("pain receptor" from Latin ''nocere'' 'to harm or hurt') is a sensory neuron that responds to damaging or potentially damaging stimuli by sending "possible threat" signals to the spinal cord and the brain. The brain creates the sens ...
s) 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
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 cau ...
,
pernio, acrorygosis,
erythromelalgia
Erythromelalgia or Mitchell's disease (after Silas Weir Mitchell) is a rare vascular peripheral pain disorder in which blood vessels, usually in the lower extremities or hands, are episodically blocked (frequently on and off daily), then become ...
, and blue finger syndrome. The diagnosis may be challenging in some cases, especially when these syndromes co-exist.
Acrocyanosis may be a sign of a more serious medical problem, such as connective tissue diseases and diseases associated with
central cyanosis
Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...
. Other causative conditions include
infection
An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...
s, toxicities,
antiphospholipid syndrome
Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. APS provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy- ...
,
cryoglobulinemia
Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures. T ...
, 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.
Signs and symptoms
Acrocyanosis is characterized by peripheral cyanosis: persistent cyanosis of the hands, feet, knees, or face.
The extremities often are cold and clammy and may exhibit some
swelling (especially in warmer weather).
The palms and soles exhibit a wide range of sweating from moderately moist to profuse, but all peripheral
pulse
In medicine, a pulse represents the tactile arterial palpation of the cardiac cycle (heartbeat) by trained fingertips. The pulse may be palpated in any place that allows an artery to be compressed near the surface of the body, such as at the nec ...
s should have normal rate, rhythm, and quality.
Exposure to cold temperatures worsens the cyanosis, while it often improves on warming.
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.
Pathophysiology
The precise mechanism of acrocyanosis is not known. The current line of thinking goes that
vasospasm
Vasospasm refers to a condition in which an arterial spasm leads to vasoconstriction. This can lead to tissue ischemia and tissue death (necrosis). Cerebral vasospasm may arise in the context of subarachnoid hemorrhage. Symptomatic vasospasm or ...
s in the cutaneous arteries and arterioles produce cyanotic discoloration, while compensatory dilatation in the postcapillary
venule
A venule is a very small blood vessel in the microcirculation that allows blood to return from the capillary beds to drain into the larger blood vessels, the veins. Venules range from 7μm to 1mm in diameter. Veins contain approximately 70% of t ...
s causes sweating. Arteriovenous subpapillary plexus shunting also occurs.
Persistent vasoconstriction at the
precapillary sphincter
A precapillary sphincter is a band of contractile mural cells either classified as smooth muscle or pericytes that adjusts blood flow into capillaries. They were originally described in the mesenteric microcirculation, and were thought to only r ...
creates a local hypoxic environment, thus releasing
adenosine
Adenosine ( symbol A) is an organic compound that occurs widely in nature in the form of diverse derivatives. The molecule consists of an adenine attached to a ribose via a β-N9-glycosidic bond. Adenosine is one of the four nucleoside building ...
into the capillary bed.
[Guyton, A.C. & Hall, J.E. (2006) ''Textbook of Medical Physiology'' (11th ed.) Philadelphia: Elsevier Saunder ] Vasospasms force adenosine to enter the capillary bed, where it vasodilates the postcapillary venules.
Such differences in vessel tone create a
countercurrent exchange
Countercurrent exchange is a mechanism occurring in nature and mimicked in industry and engineering, in which there is a crossover of some property, usually heat or some chemical, between two flowing bodies flowing in opposite directions to each ...
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.
This would seem to support case studies reporting acrocyanosis as an unusual side effect for pediatric patients taking
tricyclic antidepressant
Tricyclic antidepressants (TCAs) are a class of medications that are used primarily as antidepressants, which is important for the management of depression. They are second-line drugs next to SSRIs. TCAs were discovered in the early 1950s and wer ...
s, as these medications can inhibit the reuptake of serotonin and thus increase their blood concentrations.
Acrocyanosis has been reported in association with many other medications and substances.
Diagnosis
Acrocyanosis is diagnosed clinically, based on a
medical history
The medical history, case history, or anamnesis (from Greek: ἀνά, ''aná'', "open", and μνήσις, ''mnesis'', "memory") of a patient is information gained by a physician by asking specific questions, either to the patient or to other peo ...
and
physical examination
In a physical examination, medical examination, or clinical examination, a medical practitioner examines a patient for any possible medical signs or symptoms of a medical condition. It generally consists of a series of questions about the patien ...
; laboratory studies or imaging studies are not necessary. The normal peripheral pulses rule out
peripheral arterial occlusive disease
Peripheral artery disease (PAD) is an abnormal narrowing of arteries other than those that supply the heart or brain. When narrowing occurs in the heart, it is called coronary artery disease, and in the brain, it is called cerebrovascular disea ...
, where
arterial
An artery (plural arteries) () is a blood vessel in humans and most animals that takes blood away from the heart to one or more parts of the body (tissues, lungs, brain etc.). Most arteries carry oxygenated blood; the two exceptions are the pul ...
narrowing limits blood flow to the extremities.
Pulse oximetry
Pulse oximetry is a noninvasive method for monitoring a person's oxygen saturation. Peripheral oxygen saturation (SpO2) readings are typically within 2% accuracy (within 4% accuracy in 95% of cases) of the more accurate (and invasive) reading of ...
will show a normal
oxygen saturation
Oxygen saturation (symbol SO2) is a relative measure of the concentration of oxygen that is dissolved or carried in a given medium as a proportion of the maximal concentration that can be dissolved in that medium at the given temperature. It ca ...
. Unlike the closely related
Raynaud's phenomenon
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 cau ...
, cyanosis is continually persistent. In addition, there is usually no associated trophic skin changes, localized pain, or
ulcerations.
Capillaroscopy and other laboratory methods may be helpful but only complement clinical diagnosis in unclear cases, especially when connective tissue disorders may be present.
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
The sympathetic nervous system (SNS) is one of the three divisions of the autonomic nervous system, the others being the parasympathetic nervous system and the enteric nervous system. The enteric nervous system is sometimes considered part of th ...
to the area.
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
Calcium channel blockers (CCB), calcium channel antagonists or calcium antagonists are a group of medications that disrupt the movement of calcium () through calcium channels. Calcium channel blockers are used as antihypertensive drugs, i.e., as ...
) are effective.
Prognosis
While there is no cure for acrocyanosis, patients otherwise have excellent prognosis.
Unless acrocyanosis results from another condition (e.g. malignancy, antiphospholipid syndrome, atherosclerosis, acute ischemic limb, bacterial
endocarditis
Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves. Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or the ...
), 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.
Epidemiology
Although there is no definitive reporting on its
incidence, acrocyanosis shows
prevalence
In epidemiology, prevalence is the proportion of a particular population found to be affected by a medical condition (typically a disease or a risk factor such as smoking or seatbelt use) at a specific time. It is derived by comparing the number o ...
in children and young adults than in patients thirty years of age or older.
Epidemiological
Epidemiology is the study and analysis of the distribution (who, when, and where), patterns and determinants of health and disease conditions in a defined population.
It is a cornerstone of public health, and shapes policy decisions and evidenc ...
data suggests that cold climate, outdoor occupation, and low
body mass index
Body mass index (BMI) is a value derived from the mass (weight) and height of a person. The BMI is defined as the body mass divided by the square of the body height, and is expressed in units of kg/m2, resulting from mass in kilograms and he ...
are significant
risk
In simple terms, risk is the possibility of something bad happening. Risk involves uncertainty about the effects/implications of an activity with respect to something that humans value (such as health, well-being, wealth, property or the environme ...
factors for developing acrocyanosis.
As expected, acrocyanosis would be more prevalent in women than in men due to differences in BMI.
However, the
incidence rate
In epidemiology, incidence is a measure of the probability of occurrence of a given medical condition in a population within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time peri ...
of acrocyanosis often decreases with increasing age, regardless of regional climate. It completely resolves in many women after menopause implying significant hormonal influences.
Around 50% of patients with
POTS experience acrocyanosis of their legs while standing still.
In the newborn
Acrocyanosis is common initially after delivery in the
preterm
Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is between ...
and
full term
Pregnancy is the time during which one or more offspring develops ( gestates) inside a woman's uterus (womb). A multiple pregnancy involves more than one offspring, such as with twins.
Pregnancy usually occurs by sexual intercourse, but ca ...
newborn
An infant or baby is the very young offspring of human beings. ''Infant'' (from the Latin word ''infans'', meaning 'unable to speak' or 'speechless') is a formal or specialised synonym for the common term ''baby''. The terms may also be used to ...
.
[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 ] 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.
See also
*
Pernio (Chilblains)
*
Cyanosis
Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...
*
Peripheral artery occlusive disease
Peripheral artery disease (PAD) is an abnormal narrowing of arteries other than those that supply the heart or brain. When narrowing occurs in the heart, it is called coronary artery disease, and in the brain, it is called cerebrovascular diseas ...
*
Raynaud's phenomenon
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 cau ...
*
Postural orthostatic tachycardia syndrome
Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by an abnormally large increase in heart rate upon standing. Symptoms may include lightheadedness, brain fog, blurred vision, weakness, fatigue, headaches, heart ...
References
External links
{{Vascular diseases
Diseases of arteries, arterioles and capillaries
Medical signs
Ailments of unknown cause
Skin conditions resulting from physical factors