Raynaud Phenomenon, Erythromelalgia, and Acrocyanosis

Raynaud phenomenon is spasm of arterioles, usually in the digits and occasionally in other acral sites such as the nose and tongue. It causes intermittent skin pallor or cyanosis and is more common in females.

Erythromelalgia is a rare syndrome of paroxysmal vasodilation. It causes burning pain, increased skin temperature, and redness of the feet and, less often, the hands.

Acrocyanosis is persistent, painless, symmetric cyanosis of the hands and, less commonly, the feet. It is caused by vasospasm of small skin vessels. Its cause is unknown but may involve increased arteriolar tone with dilation of capillaries and venules.

Idiopathic Raynaud disease is most common in young women, who account for 60–90% of reported cases. Primary Raynaud syndrome commonly affects upper-limb arteries and has a good prognosis. Digital artery and arteriole vasospasm lasts minutes to hours and rarely becomes severe enough to cause gross tissue loss.

Secondary erythromelalgia is associated with:

• Myeloproliferative disorders
• Hypertension or venous insufficiency
• Diabetes mellitus, SLE, or RA
• Lichen sclerosus et atrophicus or gout
• Spinal cord disease or multiple sclerosis

Acrocyanosis usually occurs in women and is not associated with occlusive arterial disease. The digits and hands or feet remain cold and bluish, sweat profusely, and may swell. Cold intensifies the cyanosis; warming lessens it.