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

• Acrocyanosis, Secondary type

What is Secondary Acrocyanosis? (Definition/Background Information)

• Acrocyanosis is an uncommon skin condition resulting in persistent bluish discoloration of the hands or 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
• Secondary Acrocyanosis is a type of acrocyanosis that arises due to underlying factors/conditions such as exposure to cold, certain blood and vascular disorders, medications, factors causing low oxygen levels in blood, and infections
• Generally, other than the pale blue skin coloration, no other symptoms are noted. However, since Secondary Acrocyanosis is associated with an underlying condition, the symptoms and complications of the associated condition may be observed
• Blood tests, imaging studies, and a tissue biopsy may be necessary to diagnose the underlying condition associated with Secondary Acrocyanosis
• There is no cure for acrocyanosis; but, treating the underlying condition adequately may help resolve Secondary Acrocyanosis. The prognosis is dependent upon the associated condition and its severity

Who gets Secondary Acrocyanosis? (Age and Sex Distribution)

• Secondary 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 Secondary Acrocyanosis? (Predisposing Factors)

The risk factors for Secondary 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 Secondary 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. When factors leading to the causation of the condition are known or identified, it is called Secondary Acrocyanosis
• Secondary Acrocyanosis may develop from a variety of conditions including connective tissue disorders, peripheral vascular disease, infections, stress, and certain lifestyle factors

What are the Signs and Symptoms of Secondary Acrocyanosis?

The signs and symptoms of Secondary 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 arising from an underlying condition or disorder
• There is usually no pain, and in a majority of individuals there are no other symptoms other than skin discoloration

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

How is Secondary 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

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 Secondary Acrocyanosis?

In a majority of individuals, no significant complications due to Secondary 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 the underlying disorder

How is Secondary Acrocyanosis Treated?

In Secondary Acrocyanosis, undertaking treatment of the underlying condition is important. Additionally, if required, the following 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 Secondary Acrocyanosis be Prevented?

Current medical research has not established a method of preventing 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 Secondary Acrocyanosis? (Outcomes/Resolutions)

• The prognosis of Secondary Acrocyanosis is based on the severity of the underlying condition it is associated with
• Individuals with mild conditions have better prognosis than those with severe symptoms that arise from an underlying disorder

Additional and Relevant Useful Information for Secondary Acrocyanosis:

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

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