Acrocyanosis: primary or secondary form? An observational study

Acrocyanosis is the most common form of angiodystonic vascular acrosyndrome, characterized by functional microcirculatory alterations without structural vessel damage. It is traditionally classified as either primary or secondary, the latter often associated with underlying conditions.

Some observations suggest a frequent association between acrocyanosis and connective tissue diseases (CTDs). To investigate this, we conducted a study on 53 patients diagnosed with acrocyanosis: 45 females and eight males, 15 to 82 years old, with a mean age of 35 years. Secondary acrocyanosis was identified in 24 patients (45.3%).

Advanced age (≥ 40 years) was a significant risk factor for secondary acrocyanosis (relative risk = 2.5, 95% confidence interval: 1.4-4.5, p = 0.002). No significant differences were observed between sexes. CTDs were the most common conditions associated with acrocyanosis (32% of the study population and 71% of the secondary forms).

Although generally considered benign, acrocyanosis may indicate an underlying systemic disease. Clinical examination remains essential for the diagnosis of acrocyanosis. Our findings reveal a high prevalence of secondary acrocyanosis associated with CTDs. Patients with strong clinical suspicion should be referred to specialized centers for capillaroscopy and antinuclear antibody testing.