What is Acrocyanosis
Acrocyanosis is a microcirculation disorder characterized by a persistent bluish discoloration of the extremities of the body.
Acrocyanosis occurs symmetrically, especially in the hands, feet and distal parts of the face. Often, the extremities of the body are cold, sweat profusely and can swell. Unlike Raynaud's phenomenon, acrocyanosis is not easily reversible, pain is absent, there are no trophic changes or ulcerations and the peripheral arterial pulse is normal.
Pathophysiology
The disturbance is due to a constriction of the small cutaneous vessels. The consequent slowing of blood circulation locally, and the resulting desaturation of oxygen in the blood, are clinically manifested by peripheral cyanosis (bluish-purplish color of the extremities).
Causes
The exact etiology of acrocyanosis is unknown. Sometimes, the condition has been reported as a side effect of medications and other substances. Some epidemiological data suggest that cold weather, occupational exposure and low body mass index (BMI) are risk factors. Furthermore, acrocyanosis is prevalent in young women (under the age of 30) and, often, resolves completely after menopause. Vasospasm is therefore believed to be associated with neuro-hormonal abnormalities.
Primary acrocyanosis
Essential (or primary) acrocyanosis is a benign condition, sometimes associated with a neurohormonal disorder. Generally, it tends to regress spontaneously and does not require specific treatment. Emergency medical intervention, on the other hand, may be necessary if the extremities they are exposed to extreme cold for a prolonged period of time. However, acrocyanosis differs from frostbite: the latter condition is often associated with pain (the reflex pathway of thermal nociceptors warns of danger).
A number of other conditions affecting hands, feet and parts of the face, with associated skin color changes, need to be differentiated from acrocyanosis:
- Raynaud's phenomenon: reversible episodes of skin pallor of the fingers or toes, due to the constriction of small vessels exposed to cold or strong emotional stress;
- Chilblains (erythema pernio): skin irritation, caused by prolonged exposure to intense and humid cold;
- Acrorigosis: sensation of permanent and symmetrical cold in the extremities, associated with skin pallor;
- Erythromelalgia: vasodilation caused by the increase in skin temperature, which occurs with local heat, marked redness and very intense pain.
In some cases, diagnosis can be difficult, especially if these syndromes coexist.
Secondary acrocyanosis
Acrocyanosis may also be associated with a more serious health problem, which should be sought during further diagnostic work. Causal conditions include: connective tissue disease, neurological disorders, vasculitis, central cyanosis-causing problems, antiphospholipid antibody syndrome (APS). ), cryoglobulinemia, infections, toxicity and neoplasms. In these cases, the skin changes observed are known as "secondary acrocyanosis." These may have a less symmetrical distribution, are expressed at a more mature age and can cause pain and tissue damage. , the correct treatment of the underlying condition can reduce the symptoms of secondary acrocyanosis.
Signs and symptoms
Acrocyanosis is a condition characterized by persistent, symmetrical, uniform and painless peripheral cyanosis. The extremities are often cold and the skin may be edematous. Hands and feet have palmar-plantar hyperhidrosis.
Unlike the closely related Raynaud's phenomenon, cyanosis is therefore persistent. In addition, trophic skin changes, localized pains or ulcerations are usually absent.
Diagnosis
Acrocyanosis is diagnosed based on history and physical examination.
Pulse oximetry shows normal oxygen saturation. Capillaroscopy and other laboratory methods may be useful, but only to complete the clinical diagnosis in doubtful cases, especially when concomitant pathologies are suspected. In acrocyanosis, the peripheral arterial pulse is normal in terms of rhythm and quality: this allows for the exclusion of an occlusive disease of the peripheral arteries.
Treatment
There is no specific treatment for acrocyanosis and the pharmacological approach is usually useless. Among the therapeutic options, some α-adrenergic drugs and calcium channel blockers are mentioned. In extreme cases, a surgical procedure called sympathectomy is (rarely) recommended. Protection from the cold is the most effective way to avoid the onset of the disorder.
Aside from skin discoloration, there are no other symptoms and there is no loss of function, so patients with acrocyanosis can lead normal lives.
