Description

• Painless, symmetric, distal bluish discoloration usually affecting hands and occasionally feet (1)
  • Peripheral parts: Ear, nose, and nipple can be affected.
  • Affected areas can be cool.
• Often secondary to underlying cause (2)
• Unlike Raynaud phenomenon (RP), acrocyanosis is continually persistent.
  • RP has a broad differential but is distinct from acrocyanosis and is differentiated by the waxing and waning of extremity discoloration.

Epidemiology

Incidence
Unknown, uncommon
Prevalence

• Mostly affects persons <30 years old
• Typically more common in colder climates, with increased outdoor exposure, and in people with lower BMIs

Etiology and Pathophysiology

Unknown, several theories

• Vasospasm of arteries and arterioles with secondary dilatation of capillaries and veins, leading to cyanosis (2)

Genetics
Unknown

Risk Factors

• Low BMI
• Outdoor exposure in cold climates

General Prevention

• Keep hands and feet warm.
• Wear additional socks and gloves (fingerless gloves for work).

Commonly Associated Conditions

Associated conditions may occur with secondary RP and include the following:

• Connective tissue disease (systemic lupus erythematosus [SLE], scleroderma, etc.)
• Occlusive peripheral vascular disease. Buerger disease is an advanced case.
• Drugs: SSRIs, TCAs, vasopressors, α-agonists (cocaine, amphetamines), ergotamines, nicotine, some chemotherapeutic agents
• Hematologic causes: cold agglutinins, polycythemia, paraproteinemias, cryoglobulins, cryofibrinogens
• Trauma and vibration: pneumatic tools, damage to distal arteries
• Environmental exposure: chilblains (pernio), frostbite