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European Society of Cardiology > Journals > e-Journal of Cardiology Practice > E-Journal of Cardiology Practice - Volume 2

e-Journal of
Blue Toe Syndrome
Cardiology Practice An article from the e-Journal of Cardiology Practice
Vol. 2, N° 18 - 17 Jan 2004
e-Journal of Cardiology Practice
- Volume 22 Prof. Pavel Poredos , FESC

Previous volumes - e-Journal of

Cardiology Practice

e-Journal of Cardiology Practice Blue toe syndrome is one of the frequent manifestations of tissue ischaemia. It is
- Articles by Theme caused by the occlusion of small vessels, and usually occurs in elderly men who
undergo an invasive vascular procedure. Clinical manifestations of blue toe syndrome
Subscribe range from an isolated blue ischemic toe to a diffuse multiorgan systemic disease. The
treatment consists of surgical or percutaneous elimination of the source of
embolisation. Medical treatment is mostly symptomatic.

Blue toe syndrome is characterised by tissue ischaemia secondary to cholesterol crystal or

atherothrombotic embolisation. It leads to the occlusion of small vessels. Cyanosis of the digits
may have several etiologies ranging from trauma to connective tissue disease. However, the most
common cause of blue toe syndrome is atheroembolic disease or aneurysm. Embolisation occurs
typically following an ulcerated atherosclerotic plaque or aneurysm located in the aorto-iliac-
femoral arterial system. Embolisation can occur spontaneously or due to a variety of causes. Most
often, microembolisation appears in elderly men who have undergone an angiographic procedure
or vascular surgery or even anticoagulant or thrombolytic treatment. (1, 2) The differential
diagnosis of blue toe syndrome (especially secondary) includes Raynaud’s phenomenon. In
Raynaud’s syndrome, ishaemic lesions are usually more diffuse, larger areas of distal parts of
different fingers (rarely toes) are involved and are affected by vasospastic disorders. The patients
are usually younger and without any known atherosclerotic disease. In blue toe syndrome, skin
lesions are usually restricted and related to the occluded artery: fingers are rarely affected as
opposed to toes, and vasospasm is usually absent. Painful red, purple, blue or black toes can also
be seen in patients with thrombocythemia associated with polycythemia vera or in essential
thrombocythemia. (3) In these patients, ischemic lesions of fingers or toes are caused by
arteriolar inflammation and the thrombosis of microvessels that appear as a consequence.
The syndrome of cholesterol embolism is often a multiorgan disorder. Clinical presentation can
range from a cyanotic toe or livedo reticularis to a diffuse multiorgan systemic disease that can
mimic other systemic illnesses. Mild forms of the disease have a good prognosis and subside
without sequelae. However, diffuse multisystemic forms have a very poor prognosis. The kidney is
the organ that is most often affected (in approximataly 50% of cases). In the systemic form,
prognosis is poor with a mortality rate of about 70%. (4)
Blue toe syndrome is frequently misdiagnosed on initial presentation. Pedal pulses are often
palpable, which may misdirect the physician from a diagnosis of vascular pathology. The
differential diagnosis can be divided into three categories: emboli from the cardiac and arterial
system, acquired hypercoagulability disorders, and syndromes that lead to peripheral vascular
pathology. Non-invasive vascular testing, peripheral angiography, abdominal and popliteal
ultrasonography and echocardiography may be useful in discovering the origin of emboli.
Recently, transesoephageal echocardiography and MRI have been shown to be a helpful tool in
imaging the thoracic aorta and greatly detailed delineating of the anatomy of the aortic
atheroma. (5) Diagnosis is usually (definitely) confirmed with skin or muscle biopsy and
fundoscopic examination presenting cholesterol crystals.

At present, surgery (endartectomy or bypass with exclusion of the source of emboli) remains the
most viable treatment option for patients with aneurysms. In all other patients, surgery is rarely
indicated because the origin of cholesterol crystal embolisation is not certain and patients are
usually too weak for a major surgical intervention. In some of these cases, endovascular
procedures (angioplasty with stenting or endoprosthesis) can substitute reconstructive surgery in
eliminating the source of embolisation. (6, 7)
Medical treatment is mostly symptomatic: rest, warm condition, appropriate dressing, hydration,
and organ support when necessary, principally to ensure renal function. Treatment of pain that is
usually disproportionate to the extension of tissue lesion is of utmost importance. Because these
patients usually have advanced atherosclerotic disease, secondary prevention with elimination of
risk factors of atherosclerosis is mandatory. Antiplatelet drugs represent one of the basic
treatment options of blue toe syndrome. (8) The complete relief of pain and restoration of
circulation is also obtained by administration of aspirin in ischaemic complications of
polycythemia vera complete. (3) Vasodilator drugs, including alpha-1 blocking agents that are
usually used for the treatment of vasospastic disorders, have no proven efficacy in blue toe
syndrome. In diffuse and multivisceral embolisation, either colchicine or corticosteroids adjuvant
therapy may be useful. According to our experience, prostanoid drugs are also effective in
treating ischaemic lesions caused by microembolisation. In the future, large randomised studies
will be needed to help predict embolisation and, thus, decide on the proper medical therapy.

The content of this article reflects the personal opinion of the author and is not necessarily the
official position of the European Society of Cardiology.

References

1. Applebaum R. M., & Kronzon I. Evaluation and management of cholesterol embolization and the
blue toe syndrome. Curr Opin Cardiol, 1996. 11: 533-42.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
cmd=Retrieve&db=PubMed&list_uids=8889381&dopt=Abstract

2. Caldwell B. D., & Marrochello V. S. Microembolization from atheroembolic disease or aneurysm.

A case study. J Am Podiatr Med Assoc, 1996. 86: 249-52.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
cmd=Retrieve&db=PubMed&list_uids=8699345&dopt=Abstract

3. Michiels J. J., Berneman Z., Schroyens W., & Urk H. Aspirin-responsive painful red, blue, black
toe, or finger syndrome in polycythemia vera associated with thrombocythemia. Ann Hematol,
2003. 82: 153-9.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
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4. Wingo J. P., Nix M. L., Greenfield L. J., & Barnes R. W. The blue toe syndrome: hemodynamics
and therapeutic correlates of outcome. J Vasc Surg, 1986. 3: 475-80.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
cmd=Retrieve&db=PubMed&list_uids=3512857&dopt=Abstract

5. Blackshear J. L., Oldenburg W. A., & Cohen M. D. Making the diagnosis when the patient has
'blue toes'. Geriatrics, 1994. 49: 37-9, 43-5.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
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6. Renshaw A., McCowen T., Waltke E. A., Wattenhofer S. P., Tahara R. W., & Baxter B. T.
Angioplasty with stenting is effective in treating blue toe syndrome. Vasc Endovascular Surg,
2002. 36: 155-9.
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cmd=Retrieve&db=PubMed&list_uids=11951102&dopt=Abstract

7. Matchett W. J., McFarland D. R., Eidt J. F., & Moursi M. M. Blue toe syndrome: treatment with
intra-arterial stents and review of therapies. J Vasc Interv Radiol, 2000. 11: 585-92.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
cmd=Retrieve&db=PubMed&list_uids=10834489&dopt=Abstrac

8. Vayssairat M., Chakkour K., Gouny P., & Nussaume O. Atheromatous embolisms and cholesterol
embolisms: medical treatment. J Mal Vasc, 1996. 21: 97-9.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?
cmd=Retrieve&db=PubMed&list_uids=8713378&dopt=Abstract

 Notes to editor

Prof. P. Poredos
Ljubljana, Slovenia
Vice-chair of the ESC Working Group on Peripheral Circulation

The content of this article reflects the personal opinion of the author/s and is not necessarily the
official position of the European Society of Cardiology.

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