Raynaud’s phenomenon and other acral vascular syndromes

Acral vascular syndromes, including Raynaud’s phenomenon and disease, cause symptoms to manifest in the extremities (mainly in the fingers and toes), often triggered by temperature variations.

What is it?

Types of acral vascular syndrome

Discoloration that may or may not be painful is a symptom common to all acral vascular syndromes, which include:

  • Raynaud’s phenomenon and Raynaud’s disease: disorders that cause attacks in which the extremities, usually the fingers, turn white and numb when exposed to cold.
    Raynaud’s syndrome can also affect the toes, lips and tip of the nose. The disease causes episodic spasms called vasospastic attacks in which the small blood vessels in the extremities tighten more than they should when exposed to cold. These attacks usually last several minutes. After the whitening phase, the extremities may look red and “burn” as they warm up. 
  • Cold hypersensitivity in the hands and feet, also known as acrorhigosis, causes these extremities to be constantly cold and pale in cold temperatures. The condition causes little or no pain.
  • With acrosyanosis, a pain-free condition, the extremities have a purplish hue.
  • Chilblains: occurring in flare-ups, these small, very painful swollen patches on the skin have a “wrinkled” appearance and cause a burning sensation in the pulp of the toes and fingers. Chilblains are also caused by hypersensitivity to cold and often occur in the areas where shoes rub against the feet.
  • The most common warmth-triggered acral vascular syndrome is erythromelalgia: symptoms generally affect the feet and flare up on exposure to heat, which causes them to turn red and hot, combined with a burning sensation. Outbreaks typically occur in the evening in bed, on contact with the sheets. Relief can be found by taking the feet out from under the sheets and even immersing them in cold water.

Secondary forms of these syndromes also exist, associated with autoimmune diseases, diseases of the blood and blood vessels, and in the case of erythromelalgia, with nerve fiber disorders (neuropathy). It is important to systematically test for these diseases.

Diagnosis and treatment of acral vascular syndromes

The necessary exams include:

  • Clinical exam and patient history performed by a vascular medicine specialist
  • Periungual capillary microscopy performed during a vascular medicine consultation. This crucial exam is used to view the capillaries at the base of the fingernails and toenails in order to analyze the microcirculation. In the case of primary acral vascular disorders, the capillary microscopy is normal; with secondary disorders, a disarrangement of the capillary architecture is observed.
  • Blood workups to test for abnormalities and antibodies in the blood
  • Doppler ultrasound of the upper or lower limbs
  • Electromyography when a neuropathy is suspected, combined with other neurological testing

Depending on the cause, doctors may propose symptomatic treatments to relieve flare-ups, or more complex strategies in the event of an underlying disease.

Bibliography: Acrosyndromes vasculaires EM Consulte, A. Stansal.


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