Acrocyanosis

Case Presentation

A 28-year-old woman presents with progressive painless reddish-purple discoloration of both feet over one year. Symptoms started one year prior when she noticed that the creases of her toes were somewhat more purplish discolored. Six months ago she and her family noted that both her feet were diffusely more reddish-purple.  The discoloration is now present all the time when she is sitting or standing, but can be alleviated completely when lying down and lifting her feet above the level of her head.  It is independent of her surrounding temperature. She has no discoloration of her upper extremities, nose or ears; there is no increased sweating of the feet.

The worsening of symptoms led to referral to dermatology and to hematology for thrombophilia workup.  Heterozygous factor V Leiden and presence of moderate titers of anti-beta-2 glycoprotein-I IgG antibody (74 U/mL; normal < 20 U/mL) were detected. Other thrombophilia and vasculitis work-up was negative, including lupus anticoagulant, anticardiolipin antibodies, cryoglobulins, complement C3 and C4, and CH50. Venous Doppler ultrasound showed no evidence of venous thromboembolism.

On physical examination her legs below the knees are purplish-reddish discolored (images 1 and 2). The discoloration disappears on pressure (see image), but returns within 1 second after taking pressure off the involved skin area.  There is no ankle or calf swelling, but minimal puffiness of the dorsum of the left foot.  A diagnosis of acrocyanosis is made.

Pathophysiology, Treatment

Acrocyanosis is a benign disorder; a cosmetic rather than a medical issue. Typically, no treatment is needed. The etiology is not clear. This is not a thrombotic problem. There seems to be an underlying dysregulation of small blood vessel tone. One theory is that vasospasm/vasoconstriction in skin arterioles occurs first, followed by reactive vasodilatation that leads to hyperemia and then bluish discoloration due to capillary/venous sluggish flow and pooling of blood [ref 1,2]. This pathophysiological explanation supports the use of vasodilator drugs, such as calcium channel blocker, if treatment is needed, such as nifedipine 10 mg 3x/day or amlodipine 5 mg/day. For patients who are intolerant of oral calcium channel blockers because of hypotension, topical nifedipine in a cream formula made by a compounding pharmacist could be tried. Also, graduated elastic compression stockings might be worth a trial, in case there is some component of venous congestion.

APLA and Acrocyanosis

Acrocyanosis can be associated with the presence of antiphospholipid antibodies [ref 3], but as the discoloration is not due to thrombotic vascular occlusion, anticoagulation is not needed (unless, of course, the patient has another reason to be on anticoagulants). Similarly, there is no need to work up the patient with acrocyanosis for thrombophilia, as finding a thrombophilia would not have any treatment consequences.

References

1. Coffman J. Acrocyanosis and livedo reticularis. Book chapter in Raynaud’s Phenomenon. Oxford university Press, 1989.
2. Kurklinsky AK et al. Acrocyanosis: The Flying Durchman. Vascular Medicine 2011;16:288-301.
3. Diógenes MJN et al. Cutaneous manifestations associated with antiphospholipid antibodies. Int J Dermatol 2004;43:632-637.

Last updated:  Oct 14th, 2011