Acrocyanosis

Acrocyanosis can be defined as the discoloration that occurs at the level of the extremities. In the majority of the situations, the hands become cyanotic, having a characteristic bluish tinge. However, it is possible that the acrocyanosis also affects the feet and even the face (distal parts). Even though this condition was identified a long time ago, scientists are still not certain about its exact cause. As you will have the opportunity to read below, one of the most commonly incriminated causes is represented by the neurohormonal disorder. In the situation that the patient is diagnosed with the benign form of acrocyanosis, no treatment is going to be necessary.

Acrocyanosis in Toddlers

This change should not be confused with the frostbites that appear after prolonged exposure to cold temperatures. The main difference between acrocyanosis and frostbite is pain. There are numerous other conditions that can mimic the symptomatology of acrocyanosis, such as Raynaud’s phenomenon, the blue finger syndrome or erythromelalgia.

It is possible that acrocyanosis appears in association with more severe medical problems, such as the connective tissue disease. In the situation that the acrocyanosis is caused by an underlying medical condition, such as cancer or infection, it is presented as secondary acrocyanosis. Acrocyanosis can be found in newborns, whether they are preterm or born on term. Even though there is no danger associated, it is possible to administer oxygen for prophylactic reasons.

Epidemiology

This condition is more often encountered in children and young people. There are a number of factors that increase the risk for acrocyanosis, such as living in a cold climate, working in the open outdoors or having a low BMI. Given the connection with the body mass index, it should come as no surprise that acrocyanosis is more often diagnosed in women than in men. No matter the climate, the incidence of acrocyanosis decreases with age. In women, the symptoms of acrocyanosis disappear, once they have reached menopause (suggestive of hormonal influence).

Acrocyanosis Symptoms

These are the most common symptoms that appear in association with acrocyanosis:

• Peripheral cyanosis – hands, feet and face; rare situations, also the nipples
• Cold and clammy extremities
• Inflammation appears as a secondary effect of the cyanosis (especially when the temperature is not cold anymore)
• Sweating – from moderate to severe (at the level of the extremities)
• Normal peripheral pulse maintained
• Symptoms are aggravated with the exposure to cold and improved when the temperature is warmer
• Discoloration of the skin (bluish or cyanotic tinge).

Pathophysiology

The cyanosis that appears at the level of the extremities is believed to be caused by the vascular spasms in the superficial arteries of the skin. Because of these vasospasms, the small veins of the skin will dilate in a compensatory manner, which will cause the moderate-to-severe sweating at the level of the skin.

The secondary acrocyanosis is found in association with a wide range of medical conditions, such as hypoxemia (lung disease/chronic smoking), connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis, Wegener granulomatosis), cancer (ovarian cancer, Hodgkin’s lymphoma), peripheral vascular disease, malnutrition, atopic dermatitis, genetic disorders (Down’s syndrome), spinal cord injury and hematological disorders.

Diagnosis of Acrocyanosis

These are the most common methods used for the diagnosis of acrocyanosis:

• Medical history of the patient
  • When did the symptoms appear for the first time
  • Similar problems in the past
  • Current and past treatments
  • History of illnesses
  • Family medical history (genetically-inherited disorders)
• Physical examination
  • Examination of extremities
  • Identification of specific symptoms (cyanosis, sweating etc.)
• Pulse oximetry
  • Normal result in the majority of cases (oxygen saturation)
• Capillaroscopy
  • Recommended for the complementing of the clinical diagnosis
  • Often performed in patients who also suffer from disorders of the connective tissue
• Other investigations
  • Urinalysis
  • Complete blood count
  • Liver and kidney function testing
  • Antibodies
  • Imaging studies (chest X-ray, CT scan, MRI)
  • Measurement of arteriosus and venous blood gases
  • Biopsy of the skin.

The differential diagnosis is highly important in this situation, being often made with Raynaud’s phenomenon. It should be highlighted that acrocyanosis is present all the time, as opposed to the manifestations of Raynaud’s phenomenon, which can come and go (intermittent). Acrocyanosis is not accompanied by ulcerations or other changes at the level of the skin, which can be useful for the differential diagnosis as well.

Apart from the differential diagnosis, medical specialists make avail of Kroq’s sign, which can be used to guide the diagnosis in the right direction. Even though this sign is characteristic for acrocyanosis, it is important to mention that it is not also specific. In order to bring out to surface the problems related to acrocyanosis, the doctor will apply pressure to the skin. The sign is considered positive when the blood returns to the pressed area from the periphery but not from beneath.

Acrocyanosis Treatment

As it was mentioned above, in the majority of the situations, no treatment is recommended (nor available). Those who suffer from acrocyanosis are advised to avoid prolonged exposure to cold or go out wearing protective garments (gloves, hats etc.). In more severe cases, a procedure that is known as sympathectomy can be performed, in order to decrease the activity of the sympathetic nervous system at local level. Alternative treatments, such as biofeedback or hypnosis, can help with the symptoms of acrocyanosis as well.

An alternative treatment consists of the administration of vasoactive drugs but this is rarely used. Some studies have tried to demonstrate the efficacy of these vasoactive drugs for acrocyanosis but further research is necessary to confirm this. Bioflavonoids can be administered in order to improve the symptoms of acrocyanosis. New studies are performed, so as to demonstrate the efficacy of the following treatment measures for patients with acrocyanosis: nicotinic acid derivatives, hexyl nicotinate (cream) and UVB (ultraviolet light).

Prognosis

Even though acrocyanosis cannot be cured, the prognosis remains positive. The prognosis is not as good, in the situation that there is an underlying condition causing the acrocyanosis, such as cancer (associated risk of death). The overall quality of life is not affected, as the extremities continue to function normally.

Pictures of Acrocyanosis