What is Central and Peripheral Cyanosis (Polycythemia)?
Cyanosis is a medical term for when your skin, lips, or other parts of your body turn blue due to a lack of oxygen in your blood. This is not a disease itself but a sign that something else might be wrong with your body. Cyanosis typically happens if the amount of low-oxygen blood in your body increases to a certain level. It’s most noticeable in parts of your body with a lot of blood vessels near the skin, like your lips, nose, earlobes, mouth, hands, feet, and the tips of your fingers and toes.
Cyanosis usually appears when there’s an unusually low amount of oxygen in your blood, a condition known as hypoxemia. However, it’s important to note that cyanosis is not always a guaranteed indicator of hypoxemia. Since cyanosis is a symptom, careful examination is required to determine what is causing it.
Cyanosis is also divided into three types: central, peripheral, and differential.
* Central cyanosis is when your entire body and visible mucus membranes (like the inside of your mouth and nose) turn blue. This happens when your body is not getting enough oxygen due to certain conditions that increase low-oxygen blood or abnormal blood.
* Peripheral cyanosis is when only far-away parts of your body, like your hands, fingertips, and toes, turn blue. Sometimes it can also affect the areas around your mouth and eyes, but it usually doesn’t involve the mucus membranes. While peripheral cyanosis is rarely an emergency, it’s still important to find out what’s causing it and treat it quickly to avoid any complications.
* Differential cyanosis is when the upper and lower halves of your body turn blue differently. This usually signifies serious heart or lung problems.
Sometimes, you might notice a bluish discoloration in your body even when you’re getting enough oxygen. This is known as pseudocyanosis and it can occur from consuming certain drugs, toxins, or metals.
What Causes Central and Peripheral Cyanosis (Polycythemia)?
Central cyanosis, where the core of your body such as your lips or tongue turns blue, can happen because of several reasons:
1. Breathing problems due to issues with the brain, like a brain haemorrhage (bleeding in the brain), seizures, or a heroin overdose.
2. Lung-related issues which obstruct the flow of air into your lungs and the spreading of oxygen in your body, for example, asthma, a blood clot in your lungs, pneumonia, inflammation of the bronchioles (small airways in the lungs), high blood pressure in the lungs, breathing less than you should, and Chronic Obstructive Pulmonary Disease (COPD), a type of lung disease.
3. Heart problems such as heart failure, birth defects in the heart (where blood can flow from the right side of the heart to the left without being oxygenated), and diseases of the heart’s valves.
4. Conditions affecting the hemoglobin (a protein in your blood cells that carries oxygen), including too much methemoglobin (a form of hemoglobin that carries less oxygen) and sulfhemoglobin (a form of hemoglobin where sulfur has been included).
5. Having too many red blood cells (polycythemia).
6. Living at a high altitude.
7. Being too cold.
8. Having Obstructive Sleep Apnea, a condition where your throat muscles intermittently relax and block your airway during sleep.
Peripheral cyanosis, where body extremities like fingers or toes turn blue, can occur because of:
1. Heart failure or shock that decreases the amount of blood your heart pumps.
2. Narrowing of your blood vessels due to being too cold, diseases like acrocyanosis (permanently blue or purple skin) or Raynaud’s phenomenon (temporarily blue skin), or instability in the tone of your blood vessels.
3. Obstruction in the arteries reducing blood supply to certain areas, caused by diseases narrowing your blood vessels such as atherosclerosis (accumulation of fats and cholesterol in your artery walls), Buerger’s disease (inflammation and clots in your blood vessels), and atheroembolism (fat and cholesterol travelling in blood and getting stuck).
4. A block or slow flow in your veins, like a deep vein blood clot.
5. Extra thick blood due to conditions like multiple myeloma (a type of cancer), having too many red blood cells, and macroglobulinemia (a type of lymphoma).
Remember, whatever can cause central cyanosis can also cause peripheral cyanosis, with heart, lung causes and abnormalities in hemoglobin being the most common causes. Differential cyanosis, where only some parts of your body turn blue, can happen if you have a birth defect like Patent Ductus Arteriosus with high blood pressure in the lungs.
Signs and Symptoms of Central and Peripheral Cyanosis (Polycythemia)
Cyanosis is a condition where the skin turns a blue or purplish color due to a lack of oxygen in the blood. To figure out what’s causing cyanosis, healthcare providers ask about when it started, how long it lasts, whether you’ve been around any harmful substances or cold temperatures, where you’ve noticed the blue color, and any other symptoms. If you’ve had cyanosis since you were born, it means the cause is from a birth defect. But if it started more recently, it might be because of something new. It’s also important to figure out if the cyanosis is all over (central), just in your hands and feet (peripheral), or in different places (differential), because each pattern can hint toward different underlying issues.
Certain types of heart problems (called congenital heart diseases) might cause the cyanosis to start up within a week after birth or it could take longer. Fever could mean that an infection is causing your cyanosis. Sometimes, you might get clubbing (where the ends of your fingers become round and swollen) if you have certain heart problems or lung diseases. If you have blue color and trouble with breathing, it might be central cyanosis.
The healthcare provider needs good light to examine you properly. Lack of light, your skin thickness or skin color could make it difficult to see the cyanosis. It’s best to be examined at the cheeks, nose, ears and inside the mouth because the skin there is thin and has a good blood supply. This helps to figure out if cyanosis is all over your body, only in your hands and feet, or different in your extremities. The intensity of the color could show how serious your cyanosis is. Playing close attention will also help to determine if body-wide cyanosis responds to being given oxygen, which may help to rule out some specific conditions.
That examination also includes noticing the shape of your chest, how you’re breathing, and how your body and limbs are positioned. This can all help to figure out the cause of your cyanosis. Feeling your arm and leg pulses and listening to your breathing might also be helpful.
- Cyanosis in the hands and feet only
- Pink tongue since the inside of the mouth is rarely affected
- Cool hands and feet compared to warm ones in total body cyanosis
- No clubbing
- Weak pulse
- Longer than 2 seconds for color to return when the skin is pressed and then released
- Cyanosis goes away with massage and warming
- No trouble breathing
In children, some possible causes of full body cyanosis are birth defects in the heart and having too many red blood cells (polycythemia). Being in the cold and a condition called acrocyanosis are common causes of cyanosis in just the hands and feet in this age group.
Testing for Central and Peripheral Cyanosis (Polycythemia)
If you suddenly notice a blue discoloration on your body, it’s important to get it checked out quickly. While a doctor will ask about your medical history and do a physical examination, additional tests will also be needed to identify the root cause of this blue coloration, a condition medically known as cyanosis.
To check how well your body is getting oxygen, your doctor will likely use devices to measure your hemoglobin’s oxygen saturation and the partial pressure of oxygen in your blood, as well as the total hemoglobin in your blood. Different devices can do these measurements with their own distinct methods.
For instance, one simple and non-invasive way to check the oxygen levels in your blood is called pulse oximetry. It’s easily available and can be used to quickly rule out dangerously low oxygen levels, known as hypoxemia. However, this method may not give accurate results in case of peripheral cyanosis and certain blood disorders known as dyshemoglobinemias.
On the other hand, a more comprehensive test known as co-oximetry requires a sample of your arterial blood. Apart from oxygen saturation, it also measures levels of differently charged hemoglobins in your blood.
Moreover, depending on your specific circumstances, your doctor might recommend other tests. These could include checking your red and white blood cell numbers and characteristics, your blood sugar levels, possible signs of an infection, and tests related to heart and lung function. These might include an X-ray and CT scan to get more detailed images of your organs, as well as an echocardiogram to assess the structure and function of your heart.
Treatment Options for Central and Peripheral Cyanosis (Polycythemia)
Central cyanosis, a symptom characterized by blue coloring of the skin, lips, or nail beds, is treated by addressing the underlying causes. Essentially, the aim of treatment is to get to the bottom of why the symptom has emerged.
One of the first steps in treating central cyanosis is ensuring the patient has enough oxygen. This is typically done via nasal cannulas which are lightweight, easy to use devices that deliver a small, controlled amount of oxygen. In more severe cases, assisted breathing may be needed. The healthcare team may also check for and treat any metabolic abnormalities, such as low blood sugar or low calcium levels. This is particularly relevant in children who might not be eating properly due to heart diseases that cause cyanosis.
When it comes to cyanotic heart diseases, it is essential to stabilize the patient and ensure they can breathe properly. This often necessitates a referral to a Cardiologist for further assessment and possible intervention. For instances linked to birth defects of the heart, surgeries might be required. There are medications such as prostaglandin E1 which doctors might use to manage blood flow in the lungs. Other drugs, like diuretics and ACE inhibitors, could be considered for heart failure. Also, doctors would advise patients with cyanotic heart disease to take antibiotics as a precaution against bacterial endocarditis, an infection of the heart’s inner lining. Antidotes like Methylene blue can be used to counter cyanosis caused by a higher-than-normal level of methemoglobin, a type of hemoglobin that carries less oxygen.
The management of peripheral cyanosis, a symptom characterized by blue discoloration of the fingers or toes, is centered around finding and addressing the root cause. The objective is to improve blood flow and oxygen supply from the heart to various parts of the body. The symptom is usually reversible and not life-threatening, but it requires timely intervention to prevent complications.
Simple measures like gently warming and massaging the affected parts can provide relief and reverse peripheral cyanosis caused by exposure to extreme cold or Raynaud phenomenon, a condition characterized by the narrowing of small blood vessels when cold or stressed. Healthy lifestyle changes such as reducing caffeine and nicotine consumption may also be required, as these substances can cause blood vessels to constrict.
Medications that relax blood vessels, such as antihypertensive drugs, antidepressants, and drugs used for treating erectile dysfunction can be beneficial in managing peripheral cyanosis. It’s generally advisable to avoid drugs like beta-blockers, pseudoephedrine-containing medications, birth control pills, and migraine drugs, as they can constrict blood vessels. Rapid action is necessary when peripheral cyanosis is observed in extremely ill children showing signs of shock. Severely dangerous conditions such as heart failure should be managed in a hospital immediately.
An important point to note is that it takes a team of healthcare professionals with different specializations to effectively diagnose and manage the underlying causes of cyanosis.
What else can Central and Peripheral Cyanosis (Polycythemia) be?
When a doctor is trying to tell the difference between central and peripheral cyanosis, they might consider the following factors:
- Reversed differential cyanosis is more often found in the upper limbs than in the lower ones. This is seen in cases where the body’s main arteries are misaligned.
- Pigmented birthmarks
- Large tattoos
- Certain medicines, like amiodarone and silver, which can cause the skin to turn a gray-blue color as a side effect
- Blue clothing dye
- Consumption of blue-colored foods like popsicles
Possible Complications When Diagnosed with Central and Peripheral Cyanosis (Polycythemia)
Cyanosis is a symptom one can see, not a sickness itself. If not treated early enough, it can make any existing health conditions worse.
Preventing Central and Peripheral Cyanosis (Polycythemia)
It’s important for patients and their loved ones to understand what increases the risk of the illness getting worse and what actions can be taken to prevent it. Below are a few suggestions:
* Don’t smoke: Smoke damages your lungs and reduced their capacity to fight off infections.
* Keep your blood pressure in check: High blood pressure can often make the condition worse.
* Control your cholesterol level: High cholesterol can lead to other health problems that could complicate the disease.
* Avoid extreme temperatures: Extreme cold or hot weather could potentially trigger symptoms.
* Don’t stay immobile for long stretches: Moving regularly prevents your body from becoming stiff and helps maintain muscle tone, which is important for overall health.
