Acrocyanosis: Etiology, Signs, Symptoms, Treatment

Acrocyanosis is an uncommon rare condition characterized by painless, symmetrical and persistent bluish or purple discoloration of the skin and mucus membranes of the body. It mostly affects the peripheral extremities commonly involving the distal parts of feet, toes, ankles, hands and face.

Acrocyanosis prevails more in cooler geographical areas hence commonly seen in population residing in the colder climatic regions. It is also observed in people who are involved in outdoor job in such cold locations.

Acrocyanosis symptoms are seen more in asthenic females than males, and young children (newborns and preterm) as they have low body mass index (BMI).

An exact cause of acrocyanosis is still unknown. However, the most common theory proposes that the bluish skin discoloration is due to constriction or narrowing of the small arterioles followed by vasodilation in venules network of peripheral extremities, to compensate for the vasospasms in cutaneous arterial network causing hypoxia and oxygen insufficiency.

There are two types of acrocyanosis:

Primary/Essential Acrocyanosis is a benign disorder and not related to any underlying disease or condition. It is also termed as idiopathic acrocyanosis.

Secondary Acrocyanosis occurs as an outcome of an underlying or an ongoing condition, like psychiatric disorders, cardiovascular conditions mostly stroke, myocardial infarction, atherosclerosis and endocarditis etc. and neurologic or autoimmune disorders (Rheumatoid arthritis, Lupus erythematous, Neoplasms). Metabolic disorders like antiphospholipid syndrome may induce cyanosis. People suffering with nervous and psychotic disorders are at high risk of cyanosis. Depressive emotional behavior may aggravate the symptoms. It may also be associated with some concomitant medications like Amphotericin B, Clonidine, Tricyclic antidepressants, Benzocaines, Dopamine etc.

Acrocyanosis may show remissions and recurrence and usually presents with painful bluish and reddish skin discoloration over extremities and may develop ulcerations and gangrene.

The symptoms can be aggravated by cold weather and body metabolism.

Signs and symptoms of acrocyanosis include:

• Painless mottled skin
• Blue or reddish hue of skin discoloration commonly involving hands, feet, fingers and toes, nose tip, ears
• Hue differs with severity of climate and suppress with warmer climatic conditions
• Profuse sweating in the affected areas
• Cold arms and feet
• Crocq’s sign characterized by restoration of blood supply from affected area to the blanched parts due to pressure
• Normal vitals and pulse rate
• Normal body function
• Brittle nails in severe conditions.

Diagnosis is mainly based on the signs and symptoms. The other things considered while diagnosing the condition includes:

• Patient’s detailed medical history for identifying the prevailing medical conditions and ongoing medications that might precipitate acrocyanosis.
• Occupational history for eliminating the risk of geographical and climatic changes.
• No specific laboratory tests have been identified for the diagnosis.
• Oximetry helps in identifying the decreased oxygen pressure in tissues causing hypoxia.
• Doppler ultrasound of peripheral vessels is done to diagnose the condition.
• Capillaroscopy is the most appropriate diagnostic technique to study the blood flow in capillaries and their network.

The differential diagnoses which must be over-ruled for diagnosing acrocyanosis are:

• Raynaud’s syndrome/phenomenon
• Peripheral artery disease (PAD).

Acrocyanosis is believed to have an excellent prognosis. The symptoms of Acrocyanosis suppress with the warmer climate and aggravates with cooler temperatures. This condition is not associated with any severe complications of pre-existing symptoms or risk of death. There is no defined treatment for Acrocyanosis; however, precautionary weather conditions and healthy lifestyle would help prevent acrocyanosis. In case of secondary acrocyanosis, the underlying disorders would require the corresponding treatment to cure the symptoms.

Medications like vasodilator alpha blockers mainly Carvedilol, Tamsulosin etc. and calcium channel blockers like Amlodipine, Nifedipine etc. may play a major role in eliminating vasoconstriction in peripheral arteries improving the oxygen sufficiency and tissue hypoxia.

Also Read:

• Pulmonary Vascular Disease: Causes, Symptoms, Diagnosis, Treatment
• Difference Between Cardiovascular and Aerobic Exercise
• Alternative or Complementary Treatments for Vascular Dementia