Acrocyanosis

Notes

## Basic introduction

  • This is a persistent blue or cyanotic discoloration of the extremities, mainly hands, caused by vasospasm of the small vessels of the skin that is aggravated by cold.
  • It is described by some authors as a variant of Raynaud’s phenomenon[b]

## Causes of secondary acrocyanosis

  • Hypersensitivity reactions
  • Methemoglobinemia
  • Sulfhemoglobinemia
  • Chronic arsenic poisoning
  • Medicines such as SSRIs, tricyclic antidepressants, and norepinephrine
  • Malignant and benign neoplasms
  • Antiphospholipid syndrome
  • Atherosclerosis
  • Acute ischemic limb
  • Bacterial endocarditis
  • Preterm and full term newborn

## Statistics

  • It is 6-8 times more common in women than in men[c].
  • Primary acrocyanosis is mostly a disease of young adults
  • It practically resolves in women after menopause
Symptoms
  • Persistent blue or cyanotic discoloration of the extremities, mainly hands
  • Pain
  • Ulcerations and gangrene in severe cases
  • Secondary acrocyanosis often affects digits asymmetrically while primary acrocyanosis shows symmetrical distribution[a]
Diagnosis
  • Clinical review
Differential
  • Raynaud's disease
  • Pernio (chilblains or chill burns)
  • Acrorygosis
  • Erythromelalgia
Prevention
  • Preventing cold exposure
  • Keeping the feet dry
Reference

a. Kurklinsky, A. K., Miller, V. M., & Rooke, T. W. (2011). Acrocyanosis: the flying Dutchman. Vascular Medicine, 16(4), 288-301.

b. Cooke, J. P., & Marshall, J. M. (2005). Mechanisms of Raynaud’s disease. Vascular Medicine, 10(4), 293-307.

c. Merlen, J. F. (1982). Paradoxes of acrocyanosis. Adv Microcirc, 10, 95-100.

Management
  • Reassurance
  • Avoidance of cold
  • Vasodilators sometimes, though not ineffective
  • Sympathectomy (rarely required) - to relax the persistent vasospasm
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