Acrocyanosis

The term acrocyanosis is derived from the Greek words akron (meaning “extremity”) and kyanos (meaning “blue”). As a medical condition, acrocyanosis is an uncommon functional vasospastic disorder characterized by persistent bluish discoloration, primarily of the hands and feet. However, the term acrocyanosis is often used to describe diverse conditions and lacks a uniform definition. The original description of acrocyanosis is credited to Crocq in 1896, thus the eponym Crocq disease . Classically, acrocyanosis affects young females with asthenic habitus residing in regions with cooler climates. In contrast to Raynaud syndrome, the bluish discoloration of acrocyanosis is relatively persistent rather than episodic and is not associated with discomfort or triphasic color changes. Similar to Raynaud syndrome, acrocyanosis may be exacerbated by cold exposure or emotional stress. Primary acrocyanosis is not associated with an identifiable cause (i.e., idiopathic) and is usually a benign disorder with a good prognosis; skin ulceration and tissue loss are extremely rare. Primary acrocyanosis typically responds well to behavioral measures, such as avoidance of exposure to cold, and pharmacological therapy is rarely necessary. Secondary acrocyanosis occurs in association with another underlying disorder, such as a connective tissue disease or other systemic disorder. Treatment and prognosis of secondary acrocyanosis depends on the underlying condition. It often conveys a worse prognosis than primary disease, that is based upon the specific associated disorder and its severity. Acrocyanosis must be differentiated from other so-called acrosyndromes and vasoactive related disorders, such as pernio, acrorygosis, erythromelalgia, Raynaud syndrome, and livedo reticularis. In addition, an acrocyanotic appearance may be seen in conjunction with any disease that causes central cyanosis or markedly decreases blood flow to the distal extremity.