Acrocyanosis of peripheral vascular origin

Specialty: Cardiovascular.

• persistent acral cyanosis
• blue hands and feet due to vascular causes

Why it occurs

• Persistent arteriolar spasm and dilation of peripheral capillaries and venules
• Small vessel vasculitis (systemic lupus erythematosus, systemic sclerosis)
• Presence of cryoglobulins or cryoagglutinins of clinical importance
• Distal arterial occlusive disease of the extremities
• Paraneoplastic vascular syndrome secondary to hematological or solid visceral neoplasms

Initial workup

General analysis that includes complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), immunological panel (antinuclear antibodies (ANA), anti-DNA antibodies, rheumatoid factor), determination of cryoglobulins, cryoagglutinins and antiphospholipid antibodies; Periungual capillaroscopy to evaluate the density, caliber and distortion of dermal capillaries; Arterial Doppler ultrasound of distal vessels to rule out occlusive disease of large and medium vessels.

Standard management

• Nifedipine — extended-release dihydropyridine calcium antagonist to induce peripheral arteriolar vasodilation; 30 to 60 mg once daily orally, monitoring for the development of hypotension or headache
• Sildenafil — phosphodiesterase-5 inhibitor indicated in refractory cases or secondary to systemic sclerosis to improve distal tissue perfusion; 20 mg twice daily orally
• Pentoxifylline — hemorheological agent to improve the deformability of erythrocytes and facilitate microflow; 400 mg three times a day orally
• Iloprost (synthetic prostacyclin analog indicated in severe digital ischemia refractory to oral therapy; slow intravenous infusion of 0.5 to 2.0 ng/kg/min for 6 hours a day for 3 to 5 days, in a hospital setting).