Primary acrocyanosis is asymptomatic, affects digits symmetrically, and is not associated with increased morbidity or mortality. It is most often seen in patients in their 20s and 30s and completely resolves in many women after menopause. The cyanotic discoloration seen in primary acrocyanosis is due to vasospasm in the cutaneous arterioles. Cold climate, outdoor occupation, and low body mass index are risk factors for developing acrocyanosis.
Secondary acrocyanosis can be symmetrical or asymmetrical, and it is sometimes associated with pain and necrosis of the affected extremities. As opposed to primary acrocyanosis, secondary acrocyanosis results from an underlying systemic pathology. It has been associated with many etiologies, including connective tissue diseases, Buerger disease, stroke, myocardial infection, lung diseases causing hypoxia, eating disorders, hematologic disorders, neoplasms, drug exposures, genetic diseases, spinal cord injury, and infections.
I73.89 – Other specified peripheral vascular diseases
25003006 – Acrocyanosis
Differential Diagnosis & Pitfalls
- Raynaud phenomenon – paroxysmal episodes of well-demarcated cutaneous pallor and cyanosis followed by erythema
- Erythema pernio (chilblains) – rapid onset of itching, pain, tenderness after exposure to cold
- Erythromelalgia – can coexist with acrocyanosis
- Peripheral cyanosis resulting from hypoxemia or methemoglobinemia – look for cyanotic mucosae and low oxygen saturation
- Peripheral arterial occlusive disease – would have decreased pulses
- Ecchymosis – would have soft tissue swelling
- Purpura fulminans
- Leukocytoclastic vasculitis
Drug Reaction Data