A 28-year-old woman with no significant medical history presents to the clinic with bilateral, symmetric, painless, and blue discoloration of her toes that has been going on for a day. She started noticing it after running. She also notes occasional swelling and coldness of her toes bilaterally. She denies a personal or family history of blood clots, hypercoagulable disease, or connective tissue disease.
On physical examination, dorsalis pedis and posterior tibial pulses are 2+ bilaterally. Her feet and toes are nontender to examination but are cold with patchy blue discoloration up to mid foot. Upon application of pressure on the feet/toes, blanching is noted and followed by a slow and irregular return of blood from the periphery toward the center. There is no ulceration or gangrene noted. A photograph of her right foot is available (Figure 1).
Figure 1
Which one of the following is the most likely diagnosis?
Show Answer
The correct answer is: D. Acrocyanosis.
Acrocyanosis is a symmetric, painless discoloration of different shades of blue in the distal parts of the body that is marked by symmetry, relative persistence of the skin color changes with aggravation by cold exposure, and frequent association with local hyperhidrosis of the hands and feet.1 Acrocyanosis can be primary or secondary.1-3 Primary acrocyanosis is more common in younger women with no significant medical history.1 Secondary acrocyanosis should be suspected in older patients and may occur in the setting of hypoxemia, stroke, hematological disorders or malignancy, and drug/toxin exposure. On physical examination, primary acrocyanosis is nontender to palpation without ulceration or gangrene.1-3 A classic physical examination finding in acrocyanosis is Crocq's sign, which describes slow return of the blood from the periphery to the center in the area of blanching produced by pressure on the skin.2 This phenomenon is in contrast to the normal finding, which consists of return of blood both from the periphery and in the middle of the blanched area.
Peripheral artery disease (PAD) would be unlikely in this clinical scenario, as this patient had no risk factors for PAD or signs or symptoms consistent with this diagnosis.
Pernio is cold-induced skin lesions that develop after exposure to cold; they are often associated with pruritis and can be tender and painful.
Acrocyanosis is often confused with Raynaud phenomenon because both may be aggravated by cold and emotional stress; both present with discoloration of the fingers. Acrocyanosis is differentiated from Raynaud phenomenon by relative persistence of skin color changes, extension of color changes beyond the digits, symmetry, lack of pain, and absence of paroxysmal pallor.
Cutaneous manifestations in vasculitis may include urticaria, purpura, hemorrhagic vesicles, ulcers, nodules, livedo reticularis, or digital necrosis or gangrene. Her presentation was inconsistent with vasculitis.
References
Takeuchi Y, Tsukagoshi J. Primary acrocyanosis. J Gen Fam Med 2021;22:156-7.
Kurklinsky AK, Miller VM, Rooke TW. Acrocyanosis: the Flying Dutchman. Vasc Med 2011;16:288-301.
Herrick AL, Wigley FM. Raynaud's phenomenon. Best Pract Res Clin Rheumatol 2020;34:[ePub ahead of print].
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