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

• Acral Cyanosis
• Acroasphyxia
• Cyanotic Discoloration of the Extremities

What is Acrocyanosis? (Definition/Background Information)

• Acrocyanosis is an uncommon skin condition resulting in persistent bluish discoloration of the hands or feet. The term Acrocyanosis is a combination of two words - ‘acral’ meaning extremities of the body, such as the fingers and toes, and ‘cyanosis’ meaning blue or purple skin
• The mechanism of development of Acrocyanosis is not well-understood. It is believed to be due to the narrowing of small blood vessels in the hands and feet. There are two main types of Acrocyanosis:
  • Primary Acrocyanosis: When the underlying cause of the condition is unknown
  • Secondary Acrocyanosis: It arises due to underlying factors/conditions such as cold exposure, certain blood and vascular disorders, medications, smoking causing low oxygen levels, and infections
• Generally, other than the pale blue skin coloration, no other symptoms are noted. However, if Acrocyanosis is associated with an underlying disorder, then the symptoms/complications of the associated disorder may be observed
• Blood tests, imaging studies, and a tissue biopsy may be necessary to diagnose the underlying condition associated with secondary Acrocyanosis. However, examination of the presenting symptoms may be sufficient to diagnose primary Acrocyanosis
• There is no cure for Acrocyanosis; and, generally, no treatment for the primary form of Acrocyanosis may be necessary. Treating the underlying condition adequately may help resolve secondary Acrocyanosis
• The prognosis is usually excellent for primary Acrocyanosis, while the prognosis of secondary Acrocyanosis is based on the associated condition and its severity

Who gets Acrocyanosis? (Age and Sex Distribution)

• Acrocyanosis is more common in older children and young adults, although individuals of any age group are at risk. It can occur in preterm and newborn babies, but is very rare
• 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 Acrocyanosis? (Predisposing Factors)

The risk factors for Acrocyanosis may include a variety of conditions such as:

• A variety of infections
• Low body mass index; malnutrition
• Living in cold temperatures; participating in outdoor activities during cold winters
• Raynaud phenomenon
• Individuals with chilblains; a skin condition caused by exposure to cold
• Girls after puberty have a high risk for Acrocyanosis. However, the risk is very low in women after menopause
• Use of certain medications
• Trauma to the central nervous system
• Emotional and psychological stress
• Malignancies including ovarian cancer
• Certain blood disorders
• Low concentration of oxygen in blood due to a variety of factors including smoking

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 Acrocyanosis? (Etiology)

Acrocyanosis is a benign condition that probably occurs due to the constriction of blood vessels. It is considered to be a vasospastic disorder affecting the arteries. It is classified as primary and secondary based on its relation to the cause.

• If the cause is unknown, then it is called primary Acrocyanosis
• When factors leading to the causation of the condition are known, it is called secondary Acrocyanosis

Secondary Acrocyanosis may develop from a variety of conditions including connective tissue disorders, peripheral vascular disease, infections, stress, and lifestyle factors.

What are the Signs and Symptoms of Acrocyanosis?

The signs and symptoms of Acrocyanosis may include:

• Persistent bluish to purple discoloration of the hands and/or feet, more commonly the hands
• The skin discoloration may be symmetrical or asymmetrical (often noted with secondary Acrocyanosis)
• 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
• Signs and symptoms from an underlying condition, if present
• There is usually no pain, and in a majority of individuals there is no other symptoms other than the skin discoloration

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

How is Acrocyanosis Diagnosed?

Acrocyanosis may be diagnosed using the following tests and exams:

• Complete physical examination with comprehensive evaluation of medical history
• Assessment of the 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

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

In the case of secondary Acrocyanosis, diagnosis of underlying conditions resulting in Acrocyanosis is often necessary. In such cases, the following tests may be performed:

• Complete blood count (CBC)
• Urine analysis
• Liver function test
• Kidney function test
• Serum electrophoresis
• Radiographic studies of the affected region, if needed
• Pulse oximetry, to determine the level of oxygen in blood
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

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 Acrocyanosis?

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

• Cosmetic issues resulting in emotional stress
• The discoloration of skin on the extremities may remain unchanged for a long duration
• Complications arising from an underlying disorder, if any

How is Acrocyanosis Treated?

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

• For secondary Acrocyanosis, undertaking treatment of the underlying condition
• 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 and providing reassurance to the affected individual
• Symptomatic and supportive therapy, as necessary

How can Acrocyanosis be Prevented?

Current medical research has not established a method of preventing primary Acrocyanosis. However, the following measures may be considered to help lower the risk for secondary Acrocyanosis:

• Undertaking early diagnosis and treatment of any associated condition may be beneficial
• Inform your physician if you are allergic to any medication
• Avoiding exposure to cold weather and cold temperatures; use protective and warm clothing
• Managing stress, modifying certain lifestyle factors such as avoiding smoking

What is the Prognosis of 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
• The prognosis of secondary Acrocyanosis is based on the severity of the underlying condition it is associated with

Additional and Relevant Useful Information for Acrocyanosis:

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

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