Acrocyanosis in Cold Agglutinin Disease

Cold agglutinin disease is an autoimmune condition in which the immune system produces autoantibodies called cold-agglutinin that mistakenly attack red blood cells at low temperatures.

Acrocyanosis is one of the characteristic symptoms of cold agglutinin disease (CAD).

About acrocyanosis

Acrocyanosis is the bluish discoloration of the skin that occurs especially in the hands and feet. It can also affect the ears, nose, and nipples.

Acrocyanosis is caused when the blood vessels are blocked, hindering proper blood flow and resulting in a shortage of oxygen to these areas. The lack of oxygen gives the skin a bluish tinge.

There are two types of acrocyanosis — primary and secondary. The cause of primary acrocyanosis is either genetic or unknown. Secondary acrocyanosis is the result of an underlying condition such as cold agglutinin disease. It can be accompanied by pain and underlying tissue damage.

In CAD, the cold agglutinins bind to the surface of red blood cells in response to cold temperatures. As a result, the red blood cells clump together and may clog the blood vessels. This can restrict blood flow, leading to acrocyanosis.

Acrocyanosis can manifest differently based on the underlying cause. It is more common among women than men and can present at any age but is rarely reported in children.

Diagnosis of acrocyanosis

A physical examination and a detailed medical history are important for the diagnosis of acrocyanosis. The clinical diagnosis is based on the change in skin color in response to cold.

If secondary acrocyanosis due to cold agglutinin disease is suspected, a blood test can be used to detect the presence of cold-agglutinin antibodies. A nailfold capillaroscopy can also be performed to visualize the health of blood vessels under the nail bed.

Other conditions can also cause acrocyanosis. Therefore, based on the patient’s medical history, the doctors may perform additional tests to eliminate other possible conditions.

Treatment of acrocyanosis

In most cases, acrocyanosis is a harmless condition and does not require any therapeutic intervention. It can be managed by avoiding exposure to cold and warming the affected area.

Additional information

Acrocyanosis can also be associated with other conditions such as systemic lupus erythematosus and rheumatoid arthritis, and has been observed in some cancers such as lymphoma and adenocarcinoma.


Last updated: Aug. 8, 2019


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