What are the other Names for this Condition? (Also known as/Synonyms)

• Acrocyanosis, Primary type
• Idiopathic Acrocyanosis

What is Primary Acrocyanosis? (Definition/Background Information)

• Acrocyanosis is an uncommon skin condition resulting in persistent bluish discoloration of the hands and feet. The mechanism of development of acrocyanosis is not well-understood. It is believed to arise due to the narrowing of small blood vessels in the hands and feet
• Primary Acrocyanosis is a type of acrocyanosis in which the origin of the condition is unknown. This means that it is not known to be caused by any underlying disorder, infection, or medication
• Generally, other than the pale blue skin coloration, no other symptoms are noted. Examination of the presenting symptoms may be sufficient to diagnose Primary Acrocyanosis
• There is no cure for the condition; however, no treatment for Primary Acrocyanosis may be necessary for some individuals. Also, for a majority, the prognosis is typically excellent

Who gets Primary Acrocyanosis? (Age and Sex Distribution)

• Primary Acrocyanosis is common in older children and young adults, although individuals of any age group are at risk
• Generally, females (especially young adult women) are affected more than males
• No racial or ethnic group predilection is observed and the condition is observed worldwide

What are the Risk Factors for Primary Acrocyanosis? (Predisposing Factors)

• No risk factors have been presently identified for Primary Acrocyanosis
• Exposure to cold may aggravate the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Primary Acrocyanosis? (Etiology)

Primary Acrocyanosis is a benign condition of unknown cause that probably occurs due to the constriction of blood vessels. It is considered to be a vasospastic disorder affecting the arteries. 

What are the Signs and Symptoms of Primary Acrocyanosis?

The signs and symptoms of Primary Acrocyanosis may include:

• Persistent bluish to purple discoloration of the hands and/or feet; more commonly the hands are involved
• The skin discoloration may be symmetrical or asymmetrical
• The palms and soles may show increased sweating
• Rarely, it can also involve the face (ears, nose, or lips)
• The affected areas are cold to touch
• Exposure to cold temperature worsens the symptoms
• There is usually no pain, and in a majority of individuals, there are no other symptoms noted other than the skin discoloration

Note: It is usually the permanent discoloration of skin that prompts the individual to get medical attention.

How is Primary Acrocyanosis Diagnosed?

Primary Acrocyanosis may be diagnosed using the following tests and exams:

• Complete physical examination with comprehensive evaluation of medical history
• Assessment of the presenting signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood tests are required to eliminate acrocyanosis with known causes. This includes blood counts to evaluate the platelet count, clumping of red blood cells, red and white blood cell counts, and evaluation of protein and immunoglobulin levels

Skin biopsy is usually not necessary, in many cases. However, if required, a skin biopsy may be performed by the healthcare provider. Also, in a majority of individuals, no elaborate testing is needed, apart from examination of the clinical signs and symptoms.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Primary Acrocyanosis?

In a majority of individuals, no significant complications due to Primary Acrocyanosis are noted. However, in some cases, the following may be noted:

• Cosmetic issues resulting in emotional stress
• The discoloration of skin on the extremities may remain unchanged for a long duration

How is Primary Acrocyanosis Treated?

In many individuals, no treatment is necessary for Primary Acrocyanosis. If required, the following treatment measures may be considered:

• Gradually warming the affected skin region or extremities can help in improving the symptoms
• In rare cases, if the symptoms are severe, a surgical procedure called sympathectomy can be performed. It is important to note that this surgery is not performed in a vast majority of individuals
• Also, in severe cases, medications to decrease vasoconstriction may be administered
• Stress management (providing reassurance)
• Symptomatic and supportive therapy, as necessary

How can Primary Acrocyanosis be Prevented?

Current medical research has not established a method of preventing Primary Acrocyanosis.

What is the Prognosis of Primary Acrocyanosis? (Outcomes/Resolutions)

The prognosis of Primary Acrocyanosis is usually good. The condition is known to disappear during middle age in a majority of individuals.

Additional and Relevant Useful Information for Primary Acrocyanosis:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/