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Acrocyanosis

Dr. Khalid is a physician, a researcher, a health writer, and holds a Ph.D. in clinical research.

acrocyanosis

Clinical History

Acrocyanosis manifests with bluish discoloration of mucous membranes and skin of feet and hands (Wollina et al., 2018). The disease sometimes also affects nipples, lips, earlobes, and the nose. A reduction in the oxygenated hemoglobin level due to a functional abnormality in peripheral vessels triggers acrocyanosis (and its clinical manifestations). The affected patients develop bluish discoloration and sweating due to the compensatory dilatation or chronic vasospasm in postcapillary venules, capillaries, and cutaneous arteries. The medical literature documents the following two types of acrocyanosis.

  1. The idiopathic, essential, or primary acrocyanosis develops due to unspecified causes.
  2. Secondary acrocyanosis develops under the impact of a primary condition or after the prolonged administration of medications (including fluoxetine, interferon-α-2a, and imipramine). It also develops due to cardiopulmonary manifestations that trigger central cyanosis and increase the overall tissue oxygen demand.

The transient/primary acrocyanosis precipitates due to the cold environment, particularly during the winter season (Kent & Carr, 2021). Necrotizing acrocyanosis is an idiopathic condition that triggers tenderness in the toes, fingers, and cyanosis in the extremities. The extremities of the patients with acrocyanosis progressively develop gangrene or ulcers indicated by their color variations. Patients with acrocyanosis usually do not experience intense body pain.

Acrocyanosis also affects women with pre-existing cosmetic complications and reduced body mass index (Kurklinsky et al., 2011). Patients with a clinical history of anorexia nervosa, paralysis, paresis, or other neurological complications experience a high predisposition for acrocyanosis.

Clinical Examination

The history and physical of the patients with or suspected (of acrocyanosis) reveal the following results (Steinhorn, 2008).

  1. The acrocyanosis patients devoid of vascular and neurological complications rarely develop sensory manifestations or marked alteration in the quality, rhythm, or rate (of their peripheral pulses).
  2. Patients with severe acrocyanosis develop brittleness and structural irregularities in their nails.
  3. The patients with idiopathic necrotizing remittent acrocyanosis experience extremity pain and color changes.
  4. Hyperhidrosis or excessive sweating develops due to compensatory dilatation following the chronic vasospasm.
  5. A marked reduction in oxygenated hemoglobin leads to bluish discoloration in the hands and feet.

Differential Investigation/Diagnoses

Acrocyanosis may also develop as a secondary manifestation of rheumatic disease, atherosclerosis, and Buerger’s disease (Das & Maiti, 2013). The low volume pulses, gangrene, and ulcers often emanate due to thromboembolic complications in the setting of acrocyanosis. Prolonged exposure (to cold) triggers chilblains that manifest through purple discoloration of the extremities (Wollina et al., 2018). The biphasic/triphasic episodic blue, red, or white discoloration (of the digits) occurs under the impact (of Reynaud’s phenomenon) that manifests due to emotional stress and cold.

Diagnostic Assessment

The comprehensive clinical examination helps exclude perniones/Raynaud’s phenomenon and tracks/correlates the symptomology of acrocyanosis (Wollina et al., 2018). Capillaroscopy helps evaluate stasis in capillary veins of patients with acrocyanosis. The systematic evaluation of the vascular supply and morphology of venous structure is paramount to rule out connective tissue disorders in the setting of acrocyanosis.

Medical Management

The absence of standard curative treatment for acrocyanosis challenges its recovery, prognoses, or long-term outcomes. The following measures help minimize or control the symptomatology of acrocyanosis (Das & Maiti, 2013).

  1. Hygiene counseling and dietary management
  2. Reassurance and avoidance of cold exposure
  3. Use of dense insulated clothing
  4. Use of slippers and gloves
  5. Use of polypropylene liner socks due to their moisture-absorbing capacity
  6. Administration of alpha-adrenergic blocking agents or calcium channel blockers to achieve vasodilation
  7. Prescription of minoxidil or topical nicotinic acid derivatives
  8. Sympathectomy for cyanosis management
  9. Combination treatment with diltiazem and pentoxifylline

References

Das, S., & Maiti, A. (2013). Acrocyanosis: An overview. Indian Journal of Dermatology, 56(6), 417-420. doi:10.4103/0019-5154.119946

Kent, J. T., & Carr, D. (2021). A visually striking case of primary acrocyanosis: A rare cause of the blue digit. American Journal of Emergency Medicine, 40, 227.e3–227.e4. doi:10.1016/j.ajem.2020.07.064

Kurklinsky, A. K., Miller , V. M., & Rooke, T. W. (2011). Acrocyanosis: The flying Dutchman. Vascular Medicine, 16(4), 288-301. doi:10.1177/1358863X11398519

Steinhorn, R. H. (2008). Evaluation and management of the cyanotic neonate. Clinical Pediatric Emergency Medicine, 9(3), 169-175. doi:10.1016/j.cpem.2008.06.006

Wollina, U., Koch, A., Langer , D., Hansel, G., Heinig, B., Lotti, T., & Tchernev, G. (2018). Acrocyanosis: A symptom with many facets. Open Access Macedonian Journal of Medical Sciences, 6(1). doi:10.3889/oamjms.2018.035

This content is for informational purposes only and does not substitute for formal and individualized diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed medical professional. Do not stop or alter your current course of treatment. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.

© 2021 Dr Khalid Rahman

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