History

Fact Explanation
Bluish discoloration of skin & mucous membranes most often in hands and less commonly in feet. Rarely this can be also found in nose, ear lobes, lips and nipples. Acrocyanosis is caused by a functional defect in peripheral vessels which results in reduced oxygenated hemoglobin. Chronic vasospasm of cutaneous arteries & arterioles and the compensatory dilatation happens in capillaries and post capillary venules causes the sweating and bluish discoloration. There are two types in this disorder: primary acrocyanosis (essential/idiopathic), no cause can be identified even after proper investigations, secondary acrocyanosis. In the second variety there is an specific underline disease or history of certain drug consumption ( imipramine, interferon alpha 2a, fluoxetine). Two possible causes are known, first pulmonary and cardiac causes resulting in central cyanosis and second defects in local tissue oxygenation. No episodic or triphasic color changes are seen.[1,2]
Discoloration is commonly seen after exposure to cold. "Cold" is a main precipitating factor, though the underline causes vary. These changes are sometimes transient, but seen persisting in winter and even in summer.[1]
Trophic changes, ulcers and painful extremities Acrocyanosis is a painless condition usually.But this is found in necrotizing variant which is otherwise very rare. [1] Remittent idiopathic necrotizing acrocyanosis: systemic cyanotic/ erythro cyanotic color changes seen in extremities, painful tender fingers and toes, ulcers and gangrene may be found in both hands and feet.[3]
Presence of risk factors; cold climate, outdoor occupations, low body mass index (BMI) Primary acrocyanosis is common in females. It can be due to low BMI or increased cosmetic concerns. Other than this it is common in people with major neurological defects like paresis & paralytic patients. Also seen in emotionally disturbed patients who has Anorexia nervosa (low BMI). [1,4]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946
  2. KARAKAYA I, AYDOğAN M, COşKUN A, GöKALP AS. Acrocyanosis as a side effect of tricyclic antidepressants: a case report. Turk J Pediatr [online] 2003 Apr-Jun, 45(2):155-7 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12921305
  3. DAS SUDIP, ROY ALOKKUMAR, MAITI ARUNASIS. Remittent idiopathic necrotizing acrocyanosis - A rare entity. Indian J Dermatol [online] 2010 December [viewed 31 May 2014] Available from: doi:10.4103/0019-5154.60362
  4. BHANJI S, MATTINGLY D. Acrocyanosis in anorexia nervosa. Postgrad Med J [online] 1991 Jan, 67(783):33-5 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/2057423

Examination

Fact Explanation
Symmetrical distributions of painless, bluish discolorations in extremities. - This finding is frequently found in hands and less commonly in feet. This can be found rarely in nose, ear lobes, lips and nipples. Acrocyanosis is caused by a functional defect in peripheral vessels which results in reduced oxygenated Hemoglobin. Chronic vasospasm of cutaneous arteries and arterioles and the compensatory dilatation happens in capillaries and post capillary venules causes the sweating and bluish discoloration. [1]
Hyperhidrosis of hands and feet; dorsum is dry & cold, soles & planter surfaces are more wet Chronic vasospasm of cutaneous arteries and arterioles and the compensatory dilatation happens in capillaries and post capillary venules causes the sweating [1]
Trophic changes, ulcers, gangrenes, swollen, tender extremities Remittent idiopathic necrotizing acrocyanosis: systemic cyanotic/ erythro cyanotic colour changes seen in extremities, painful tender fingers and toes, ulcers and gangrene. [2]
Nail changes: irregular, brittle nails This changes are seen in severe cases. [1]
No sensory changes, Normal peripheral pulses in rate, rhythm & quality [1] Important negative findings that exclude neurological and vascular causes.
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946
  2. DAS SUDIP, ROY ALOKKUMAR, MAITI ARUNASIS. Remittent idiopathic necrotizing acrocyanosis - A rare entity. Indian J Dermatol [online] 2010 December [viewed 31 May 2014] Available from: doi:10.4103/0019-5154.60362

Differential Diagnoses

Fact Explanation
Acrosyndromes : Raynaud's phenomenon Raynaud's phenomenon causes episoidic triphasic/ biphasic discolorations (white, red & blue) in digits [1]. This condition is triggered by cold and emotional stress. [2]
Acrosyndromes: Pernios/Chilblain Pernios occurs following exposure to cold. Digits become erythematous and purple. Itching, burning and pain are prominent. Swollen and tender digits are common. In severe disease blisters, pustules and ulcers are seen. [1]
Vaso- occlusive disorders Conditions such as Buerger's disease, atherosclerotic disease, Rheumatic disease and other collagen vascular disorders, can cause symptoms similar to acrocyanosis. In thromboembolic diseases pulses are of low volume or are absent. Ulcers and gangrene are prominent. [1,3]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946
  2. KURKLINSKY A. K., MILLER V. M., ROOKE T. W.. Acrocyanosis: The Flying Dutchman. Vascular Medicine [online] December, 16(4):288-301 [viewed 31 May 2014] Available from: doi:10.1177/1358863X11398519
  3. JORGE VC, ARAúJO AC, NORONHA C, PANARRA A, RISO N, VAZ RISCADO M. Buerger's disease (Thromboangiitis obliterans): a diagnostic challenge. BMJ Case Rep [online] 2011 Sep 13 [viewed 01 June 2014] Available from: doi:10.1136/bcr.08.2011.4621

Investigations - for Diagnosis

Fact Explanation
Primarily by a clinical diagnosis Take a focussed history and careful examination will reveal the diagnosis. Raynaud's phenomenon and Perniosis should be excluded. [1]
Capillaroscopy This visualizes venous stasis in capillaries. Capillaroscopy is the only investigation used in practice to diagnose acrocyanosis. [1] This is used to identify capillary morphology, distribution and blood flow. It helps to differentiate primary acrocyanosis from early stage connective tissue disorders. [2]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946
  2. KURKLINSKY A. K., MILLER V. M., ROOKE T. W.. Acrocyanosis: The Flying Dutchman. Vascular Medicine [online] December, 16(4):288-301 [viewed 31 May 2014] Available from: doi:10.1177/1358863X11398519

Investigations - Screening/Staging

Fact Explanation
Antinuclear factor, Rheumatoid factor, Cardiolipin antiboby, Lupus antibody These are done if there is suspicion of a connective tissue disorder. [1]
Antiphospholipid antibody To exclude Antiphospholipid syndrome.[1]
Leucocyte oligosaccharide enzyme This is done to analyse alpha-L-fucosidase which is deficient in fucosidosis [1]
Imaging: CT scan, MRI scan Useful only to exclude underlying neoplastic disorders, spinal cord injury & nerve plexus neuropathy. [1]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946

Management - General Measures

Fact Explanation
Reassurance Explain to the patient that the color change of fingers doesn't indicate any thing sinister. Primary acrocyanosis is a benign disease. Though there is no cure, prognosis is good and there is no increase in risk of death. When the disease is mild no medical treatment is necessary. [1]
Avoid exposure to cold- - Cold is a major precipitating factor. Advise patients to use insulated clothing, gloves, socks and slippers. [1]
Life style modification, Advise of smoking cessation and implementing dietary changes. [1]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946

Management - Specific Treatments

Fact Explanation
Calcium channel blockers This drug has a vaso dilatory effect. Though not very effective; nifidifine and diltiazam has been used with some improvement of symptoms. [1]
Beta blocking agents These drugs block the catecholamine action. Acrocyanosis patients have reported some improvement. [2]
Topically applied nicotinic acid derivatives and minoxidil These are used in clinical trials with some improvement in symptoms, but the responses were greatly variable. [2]
Sympathectomy This is rarely used in very severe cases.[2,3]
Treatment of the underlying cause in secondary acrocyanosis This varies according to the cause. Some of the causes are: hypoxemia, myocardial infarction, stroke, buerger's disease, pulmonary hypertension and drugs such as Amphotericin B, sirolimus, tricyclic anti depressents, interferon, IV immunoglobulins. Thus, the management will vary according to the etiology.[2]
References
  1. DAS SUDIP, MAITI ARUNASIS. Acrocyanosis: An overview. Indian J Dermatol [online] 2013 December [viewed 30 May 2014] Available from: doi:10.4103/0019-5154.119946
  2. KURKLINSKY A. K., MILLER V. M., ROOKE T. W.. Acrocyanosis: The Flying Dutchman. Vascular Medicine [online] December, 16(4):288-301 [viewed 31 May 2014] Available from: doi:10.1177/1358863X11398519
  3. SHEPPERD NL. Acrocyanosis treated by Sympathectomy. Proc R Soc Med [online] 1936 Mar, 29(5):524 [viewed 01 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19990660