Acrocyanosis (benign)
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{{DiseaseDisorder infobox |
Name = [[Acrocyanosis]] |
ICD10 = {{ICD10|I|73.8| |i|00}} |
ICD9 = {{ICD9|443.89}} |
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DiseasesDB = 29444 |
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'''Acrocyanosis''' refers to a persistent blue or [[cyanosis|cyanotic]] discoloration of the [[digits]], most commonly occurring in the hands although also occurring in the face and feet as well.
<ref name="Cecil 1">Olin, J.W. (2004). Other peripheral arterial diseases. In L. Goldman & D. Ausiello (Eds.), ''Cecil Textbook of Medicine, 22nd Edition''. (Vol 1, pp. 475). Philadelphia: WB Saunders ISBN 0-7216-9653-8</ref>
<ref name="internal medicine">Creager, M.A. & Dzau, V.J. (2005). Vascular diseases of the extremities. In D.L. Kasper, A.S. Fauci, D.L. Longo, E. Braunwald, S.L. Hauser, & J.L Jameson (Eds.), ''Harrison's Principles of Internal Medicine'' (16th ed., pp. 1490). New York: McGraw-Hill ISBN 0071402357</ref>
<ref name="Merck"> (2006). Peripheral vascular disorders. In R.S. Porter, T.V. Jones, & M.H. Beer (Eds.), ''Merck Manual of Diagnosis and Therapy'' (18th ed., ch. 212). New York: Wiley, John & Sons ISBN 0911910182</ref> The principle form of acrocyanosis is that of a [[benign]] cosmetic condition<ref name="Cecil 1"/>, sometimes caused by a relatively benign [[neurohormone|neurohormonal]] disorder.<ref name="internal medicine"/> Regardless of its cause, the benign form typically does not require medical treatment. A [[medical emergency]] would ensue if the extremities experience prolonged periods of exposure to the cold, particularly in children and patients with poor general health.<ref name="internal medicine"/> However, [[frostbite]] differs from acrocyanosis because pain (via thermal [[nociceptor]]s) often accompanies the former condition, while the latter is very rarely associated with pain.
[[Acrocyanosis]] may be a sign of a more serious and larger medical problem, such as connective tissue diseases and diseases associated with [[central cyanosis]].<ref name="Cecil 1"/>
===Incidence, Prevalence, and Epidemiology===
Although there is no definitive reporting on its [[incidence (epidemiology)|incidence]], acrocyanosis shows [[prevalence]] in children and young adults than in patients thirty years of age or older.<ref name="internal medicine"/> [[Epidemiology|Epidemiological]] data suggests that cold climate, outdoor occupation, and low [[body mass index]] are significant [[risk]] factors for developing acrocyanosis.<ref name="Carpentier">{{cite journal | author=Carpentier, P.H. | title=Definition and Epidemiology of Vascular Acrosyndromes | journal=Rev Prat | volume=48 | issue=15 | pages=1641–6 | year=1998 | pmid=9814064}}</ref> As expected, acrocyanosis would be more prevalent in women than in men due to differences in BMI.<ref name="Merck"/> However, the [[incidence rate]] of acrocyanosis often decreases with increasing age, regardless of regional climate.<ref name="Carpentier"/> Case reports have found acrocyanosis to be more prevalent in patients with [[autism|autistic]] disorders such as [[Asperger's Syndrome]].<ref name="Carpenter">{{cite journal | author=Carpenter, P.K., & Morris, D. | title=Association of Acrocyanosis with Asperger's Syndrome | journal=J Ment Defic Res | volume=Feb, 34 | issue=23 | pages=87–90 | year=1990 | pmid=2325122}}</ref>
===Pathophysiology===
The precise [[etiology]] of acrocyanosis is unknown. The current line of thinking goes that [[vasospasm]]s in the cutaneous arteries and arterioles produce cyanotic discoloration, while compensatory dilatation in the postcapillary [[venule]]s causes sweating.<ref name="Cecil 1"/><ref name="Merck"/> Persistent vasoconstriction at the [[precapillary sphincter]] creates a local hypoxic environment, thus releasing [[adenosine]] into the capillary bed.<ref name="Medical Physiology">Guyton, A.C. & Hall, J.E. (2006) ''Textbook of Medical Physiology'' (11th ed.) Philadelphia: Elsevier Saunder ISBN 0-7216-0240-1</ref> Vasospasms force adenosine to enter the capillary bed, where it vasodilates the postcapillary venules.<ref name="Medical Physiology"/> Such differences in vessel tone create a [[countercurrent exchange]] system that attempts to retain heat. Profuse sweating would then be caused by an overwhelmed countercurrent exchange system. In addition to adenosine, other hormones may contribute to acrocyanosis such as increase blood levels of serotonin.<ref name="Carpenter"/> This would seem to support case studies reporting acrocyanosis as an unusual side effect for pediatric patients taking [[tricyclic antidepressant]]s, as these medications can inhibit the reuptake of serotonin and thus increase their blood concentrations.<ref name="Karakara">{{cite journal | author=Karakara, I., Aydoğan, M., Coşkun, A., & Gökalp, A.S. | title=Acrocyanosis as a side effect of tricyclic antidepressants: A case report | journal=The Turkish Journal of Pediatrics | volume=45 | pages=155–57 | year=2003}}</ref>
===Clinical Manifestations and Presentation===
Acrocyanosis is characterized by peripheral cyanosis: persistent cyanosis of the hands or of the hands, feet, or face.<ref name="Merck"/> The extremities often are cold and clammy and may exhibit some [[edema|swelling]].<ref name="Cecil 1"/> The palms and soles exhibit a wide range of sweating from moderately moist to profuse, but all peripheral [[pulse]]s should have normal rate, rhythm, and quality.<ref name="internal medicine"/><ref name="Merck"/> Exposure to cold temperatures worsens the cyanosis, while it often improves on warming.<ref name="Cecil 1"/> Patients normally are asymptomatic and therefore there is usually no associated pain; the most common sign, discoloration, usually is what prompts patients to seek medical care.<ref name="internal medicine"/><ref name="Merck"/>
===Diagnosis===
Acrocyanosis is diagnosed clinically, based on a [[medical history]] and [[physical examination]]; laboratory studies or imaging studies are not necessary. The normal peripheral pulses rule out [[peripheral arterial occlusive disease]], where [[artery|arterial]] narrowing limits blood flow to the extremities. [[Pulse oximetry]] will show a normal [[oxygen saturation]]. Unlike the closely related [[Raynaud's phenomenon]], cyanosis is continually persistent. In addition, there is usually no associated trophic skin changes, localized pain, or [[ulcer]]ations.<ref name="Cecil 1"/><ref name="Merck"/>
===Treatment===
There is no standard medical or surgical treatment for acrocyanosis, and treatment, other than reassurance and avoidance of cold, is usually unnecessary. The patient is reassured that no serious illness is present. A [[sympathectomy]] would alleviate the cyanosis by disrupting the fibers of the [[sympathetic nervous system]] to the area.<ref name="internal medicine"/> However, such an extreme procedure would rarely be appropriate. The same effect could be accomplished with [[alpha blocker|α-adrenergic blocking agents]] or [[Calcium channel blockers|caclium channel blockers]].<ref name="Cecil 1"/>
===Prognosis===
While there is no cure for acrocyanosis, patients otherwise have excellent prognosis.<ref name="Cecil 1"/> Unless the condition is not benign, there is no associated increased risk of disease or death, and there are no known complications. Aside from the discoloration, there are no other symptoms: no pain, and no loss of function. Patients can expect to lead normal lives.
===Newborn Considerations===
Acrocyanosis is common initially after delivery in the [[premature birth|preterm]] and [[full term]] [[newborn]]<ref name="Pediatrics">Engle, W.A. & Boyle, D.W. (2005). Delivery room management and transitional care. In L.M. Osborn, T.G. DeWitt, L.R. First, & J.A. Zenel (Eds.), ''Pediatrics'' (pp. 1250-61). Philadelphia: Elsevier Mosby ISBN 0-323-01199-3</ref> Intervention normally is not required, although hospitals opt to provide supplemental oxygen for precautionary measures.
==See also==
* [[Acrocyanosis|Acrocyanosis (not benign)]]
* [[Chilblains|Pernio (Chilblains)]]
* [[Cyanosis]]
* [[Peripheral artery occlusive disease]]
* [[Raynaud's phenomenon]]
==References==
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==External links==
*[http://www.merck.com/mmpe/sec07/ch080/ch080b.html Acrocyanosis]. ''The Merck Manual''.
[[Category:Cardiovascular diseases]]
[[Category:Medical signs]]
[[Category:Ailments of unknown etiology]]
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