What to know about acrocyanosis

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Acrocyanosis refers to a blood vessel disorder that can result in skin discoloration. It occurs when blood vessels narrow, causing skin color changes that often present in the fingers and toes.

Crocq first described acrocyanosis in 1896, which is why some may refer to it as Crocq’s disease. The name derives from the Greek words “akron” (extremity) and “kyanos” (blue), which describe the classic presentation of bluish discoloration of the extremities.

There are two types of acrocyanosis: Primary and secondary. Primary acrocyanosis, also known as idiopathic or essential acrocyanosis, occurs when no underlying condition is present. Secondary acrocyanosis occurs in association with another condition or after administering certain drugs.

Acrocyanosis is a type of cyanosis, which simply refers to the bluish discoloration of skin due to a lack of oxygen-rich blood. Specifically, acrocyanosis is a type of peripheral cyanosis, which describes when cyanosis affects the extremities.

The bluish discoloration occurs due to a reduction of hemoglobin in the blood. Hemoglobin is a molecule present in red blood cells that allows them to carry oxygen throughout the body. Hemoglobin is also responsible for the red color of these cells.

Multiple factors can influence acrocyanosis. Generally, acrocyanosis occurs due to blood vessels narrowing, which causes a reduction in blood flow to the extremities.

The exact cause of primary acrocyanosis is often unknown, which is why health experts may refer to it as idiopathic acrocyanosis. Factors that can contribute to secondary acrocyanosis include:

• lung conditions
• arterial diseases
• connective tissue diseases
• blood conditions
• infections
• certain drugs

The characteristic feature of acrocyanosis is skin discoloration. Often, this may present as a bluish discoloration that can have different hues, such as bluish-pink or brownish-yellow.

Another feature often present with acrocyanosis is Crocq’s sign. This refers to the slow and irregular return of blood to an area of blanching, or pale skin, after applying pressure.

Typically, acrocyanosis affects the extremities, particularly the fingers and toes. However, symptoms can also appear on different parts of the body and may extend to include hands, feet, forearms, and face. Other possible symptoms may include:

• excessive sweating of the hands and feet
• cold hands and feet
• swelling of digits
• worsening of symptoms due to cold or emotional stress
• improvement of symptoms with rewarming

It is not uncommon for newborns to present with acrocyanosis for the first few hours of life. This is due to blood and oxygen first circulating to the most important areas of the body, such as the brain, lungs, and kidneys, rather than the extremities.

This typically resolves as the baby’s body becomes familiar with the new blood circulation pattern. However, it can return if the baby is cold, such as after a bath. This is also normal and should resolve by itself.

Symptoms may also present slightly differently between primary and secondary acrocyanosis. For example, with primary acrocyanosis, symptoms are usually symmetrical, affecting both hands or both feet. However, with secondary acrocyanosis, symptoms often only affect one side.

Vs. Raynaud’s phenomenon

Raynaud’s phenomenon refers to an extreme narrowing of the blood vessels, which causes discoloration of the digits. Symptoms can also worsen in response to cold and emotional stress, and may also present with excessive sweating.

As such, it is not uncommon to confuse the conditions. Typically, a health expert may be able to differentiate them due to the persistence of skin color changes. Acrocyanosis usually presents with continual color changes, while Raynaud’s presents with paroxysmal pallor, which refers to a sudden recurrence or intensification of symptoms, typically in response to a trigger such as cold weather.

To diagnose acrocyanosis, a doctor will initially ask about a person’s medical history and symptoms, then conduct a physical exam. They will check the affected area for signs of persistent discoloration, cold temperature, and sweating.

A doctor may order further tests to help distinguish from other conditions that also cause bluish extremities, such as Raynaud’s phenomenon. This may include a capillaroscopy. This is a noninvasive technique that measures the circulation in the small blood vessels of the nail bed.

If they suspect secondary acrocyanosis, they will likely order further laboratory and imaging tests that can help determine the underlying condition.

In many cases, people may not require treatment for acrocyanosis.

Primary acrocyanosis is often harmless. It may only require conservative approaches to help manage symptoms. For example, this may include behaviorial changes, such as trying to avoid exposure to colder temperatures.

Evidence suggests that medications, such as calcium channel blockers and beta-blockers, are typically ineffective for primary acrocyanosis.

Treatment for secondary acrocyanosis will involve treating the primary cause. For example, if a person is experiencing drug-induced acrocyanosis, then symptoms will improve after stopping the causative drug. A person can discuss options for changing medications with their healthcare professional.

Acrocyanosis describes skin discoloration of the extremities, often occurring in the fingers and toes. The discoloration is often blue, but it can have different hues, such as brownish-yellow. It typically occurs due to a narrowing of blood vessels, which causes a reduction in blood flow to the extremities.

Primary acrocyanosis is usually harmless and may not require treatment. Secondary acrocyanosis can be serious, depending on the underlying cause. If a person experiences symptoms of acrocyanosis, it is advisable to contact a doctor. They can determine whether it is occurring due to an underlying condition that requires treatment.