Acrocyanosis is a persistent dusky or blue painless discoloration of hands, feet and less commonly the face. It is commonly seen during winter months and is accentuated by cold exposure and is frequently associated with local hyperhidrosis of hands and feet. Cyanosis of the hands and feet increases as the temperature decreases. Acrocyanosis commonly starts in adolescence and may persist into adult life, while in some individuals it may spontaneously remits.
This condition is most probably a vascular defect where there is constriction of peripheral arterioles when exposed to low temperature. This results in decrease blood flow to the peripherals. It is usually associated with painless mottled duskiness of both hands and feet. Less frequently it can also affect facial areas like tip of nose and ears. Trophic changes like ulceration are very rare. It may sometimes be confused with Raynaud phenomenon which is usually associated with pain and is often episodic and segmental. In case of acrocyanosis secondary to systemic disorder, it often affects digits asymmetrically and may be associated with pain and tissue loss. The skin changes in acrocyanosis may be temporary after cold exposure but usually persist during the winter and even throughout the summer months.
What causes Acrocyanosis?
Acrocyanosis is genetically determined and usually starts in adolescence. In some cases the cause is unknown while in some cases it may be secondary to several systemic disorders which are listed below. When it develops for the first time during adulthood, it is very important to find out the secondary cause. In few cases it may be induced by drugs like Butyl nitrate, Interferon-alfa (2a) etc.
Acrocyanosis due to systemic disorders
Connective tissue disorders
Primary and secondary antiphospholipid antibody syndrome
Benign and malignant paraproteinaemias
Paraneoplastic syndrome
Cold agglutinin disease
Cryoglobulinaemia
Anorexia nervosa
Bulimia nervosa
Chronic orthostatic intolerance
Postural orthostatic tachycardia syndrome of adolescents
Adolescent chronic fatigue syndrome
Brachial plexus neuropathy
Chronic arsenic poisoning
Fucidosis
Ethylmalonic encephalopathy
Mental retardation
Schizophrenia
Essential thrombocythaemia
Treatment of Acrocyanosis
There is no effective medical treatment for acrocyanosis. Supportive measures to keep the skin warm are helpful. The commonly affected areas like hands are feet should be protected with woolen clothing throughout the winter season. Heating pads may be used to warm the peripherals. Smoking should be strictly avoided. As central cooling triggers peripheral vasoconstriction, keeping the whole body warm is very important. If acrocyanosis was due to drugs, stopping the drug will improve the condition. Treatment with vasodilator medications have been tried with limited success. In case of secondary acrocyanosis, treatment of underlying systemic cause may improve the condition.