Acrocyanosis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Acrocyanosis is a peripheral vascular disorder characterized by bluish or cyanotic skin discoloration due to decreased oxyhemoglobin, known as the reduced oxygen level in hemoglobin. The most common area is the extremities which include the hands and feet. Acrocyanosis in adults is a rare condition, as this condition more commonly affects newborns.

What Is Acrocyanosis?

Acrocyanosis is a functional peripheral vascular disorder characterized by deep blue or dusky mottled skin discoloration in extremities such as hands and feet; sometimes, discoloration is also seen on the face. Acrocyanosis occurs due to the vasospasm of peripheral vessels caused due to decreased oxyhemoglobin. Acrocyanosis is of two types:

1. Primary or Idiopathic Acrocyanosis:

This is caused due to small blood vessel constriction, which reduces blood flow to the hands and feet. This can be caused due to

• Cold temperatures.

• Surviving in higher altitudes with more wind, cold, and reduced oxygen pressure.

• Any genetic condition that impacts blood vessels.

2. Secondary Acrocyanosis:

This type is caused due to some underlying condition. The symptoms of acrocyanosis are the first signs of the underlying condition. Secondary acrocyanosis caused are;

• Vascular diseases.

• Infection.

• Blood condition.

• Solid tumor.

• Genetic conditions.

• Blood disorders.

Who Gets Acrocyanosis?

Acrocyanosis is found to be more susceptible in females than in males. Primary acrocyanosis is present in young adults between 20 to 30 years of age. Acrocyanosis is a rare condition seen in post-menopausal women and children. Sometimes acrocyanosis is found in association with Raynaud's phenomenon and erythromelalgia. Acrocyanosis is most commonly seen in anorexia nervosa and emotionally and mentally disturbed people.

• Females are more susceptible to acrocyanosis than males.

• Young adults at 20 to 30 years of age.

• Post-menopausal women and children.

• Infants.

• Smokers.

• People with anorexia nervosa.

• Emotional and disturbed people.

What Causes Acrocyanosis?

Primary Acrocyanosis, idiopathic or essential acrocyanosis, has no underlying cause. Secondary acrocyanosis occurs in specific conditions. For example, Acrocyanosis can occur due to psychiatric, autoimmune, infective neurologic, metabolic, and other reasons. In addition, central oxygenation defects in certain lung and heart conditions and local defects in any vascular tissue can cause acrocyanosis. Drugs like tricyclic antidepressants or TCA intake with the action of vasoconstriction can also cause acrocyanosis in pediatric patients. The various other cause of acrocyanosis is as follows:

• Hypoxemia in smoking individuals.

• Cold exposure.

• Neoplastic conditions such as Hodgkin's lymphoma (a form of cancer that attacks the lymphatic system) and ovarian cancer.

• Connective tissue disorders like

• Rheumatoid arthritis is an autoimmune condition where the immune system attacks the body’s healthy cells.

• Lupus erythematosus is an inflammatory condition where the immune system attacks its own body's tissue.

• Overlap syndrome combines systemic lupus erythematosus, dermatomyositis, and progressive systemic sclerosis.

• Granulomatosis (blood vessel inflammation) with polyangiitis.

• Peripheral vascular disease.

• Blood disorders.

• Malnutrition.

• Eating disorders.

• Toxins and drug intake such as tricyclic depressants.

• Any infection.

• Dermatitis, particularly atopic dermatitis.

• Genetic disorders like

  • Mitochondrial disease is a genetic condition that affects the energy production by mitochondria.
  • Down syndrome, this condition is caused due to an extra chromosome which causes developmental delays.
  • Ehlers-Danlos syndrome is a group of inherited conditions affecting the skin, joints, etc.

• Infancy.

• Buerger's disease affects the blood vessels of the hands and legs.

• Injury to the spinal cord.

• Anorexia nervosa is an eating condition that makes people worry about their weight.

• Atrophic rhinitis.

• Any psychiatric conditions.

• Physiological factors.

What Are the Clinical Features of Acrocyanosis?

• Acrocyanosis shows dusky discoloration of the hands and feet. sometimes discoloration occurs less frequently in the feet.
• Acrocyanosis features are also seen in peripheral areas like the ear, nose, nipple, and lips.
• Extremities will be cold and clammy, associated with swelling. Sweating is observed in the palms and soles.
• Acrocyanosis is painless.
• The skin is often due to the presence of local edema.
• Visible swelling in fingers showing a shiny appearance. Acrocyanosis also causes ulceration and gangrene in fingers due to increased susceptibility to cold and pain.
• Sensory changes are absent.
• Pulse rate, rhythm, and quality will be normal.
• The nails will be brittle and irregular in severe cases.
• Acrocyanosis also shows other features such as
  • Erythrocyanosis is a condition that results in reddish-blue discoloration of the skin caused due to exposure to cold temperatures.
  • Perniosis a small blood vessel inflammation caused due to exposure to cold.
  • Livedo reticularis is a reddish-blue discoloration of the skin.

What Are the Symptoms Associated With Acrocyanosis?

The common acrocyanosis symptoms are

• Blue-colored extremities like hands and feet.

• Clod and sweaty extremities.

• Lower skin temperature.

• Cold, clammy, swelling, sweating, edema, ulcer, and sometimes gangrene.

Symptoms due to transient cold exposure will be reduced with warming. Chilblain (tiny itchy swelling over the skin caused due to cool temperature) or perniosis (small blood vessel inflammation caused due to cold temperature) with red and purple discoloration in digits after cold exposure. Itching, burning, and pain are absent in acrocyanosis.

Do Babies Have Acrocyanosis?

Acrocyanosis is very often in newborn babies. Acrocyanosis is a common condition in healthy infants and persists for one to two days after birth. By warming the temperature, the symptoms of acrocyanosis get resolved. As long as cyanotic symptoms are absent in the center part of the body, acrocyanosis is normal in babies.

How to Diagnose Acrocyanosis?

• Acrocyanosis is diagnosed by a good clinical history and physical examination of the patient.

• Hematological findings are essential for lymphoproliferative disease and thrombocytopenia.

• Depending on the signs and symptoms, laboratory investigations and imaging studies are needed to find the cause of secondary acrocyanosis.

• Secondary acrocyanosis due to connective tissue disorders is screened for antinuclear factor, rheumatoid factor, cardiolipin antibody, and lupus antibody.

• Neoplastic etiology is diagnosed with a computer scan or CT, magnetic resonance imaging or MRI, and nerve conduction velocity or NCV.

• Acrocyanosis is clinically diagnosed by capillaroscopy to visualize the blood capillaries in venous stasis.

• The four sufficient symptoms to diagnose acrocyanosis are cyanotic discoloration of extremities, local hypothermic condition, sweating, and rigid digit.

• Acrocyanosis is diagnosed by:

  • Good history-taking.

  • Physical examination.

  • A hematogram in lymphoproliferative disease and thrombocytopenia is a complete blood count test which is a combination of a set of medical laboratory tests that provide results of the total number of cells present in a person’s body.

  • Pulse oximetry is a monitor which measures the oxygen saturation level in the blood of a person.

  • Nailfold capillaroscopy is used to determine the microcirculation and identify pathological alterations. Specialized microscopy is used to view through the epidermis of the nail fold and examine the capillaries.

  • Skin biopsy if needed.

  • Computed scan or CT, magnetic imaging resonance or MRI, nerve conduction velocity or NCV for neoplastic conditions and spinal cord injuries.

  • Complete blood count or CBC.

  • Chest X-ray.

What Is the Treatment for Acrocyanosis?

Acrocyanosis has no standard treatment. Acrocyanosis has no medical or surgical curative methods. Medications are not recommended in mild cases of acrocyanosis. The bluish discoloration is not a sign of any severe condition.

• Lifestyle modifications, a proper healthy diet, avoiding cold exposure, and reassurance can be done.

• In cold exposure, thick insulating clothing can be used.

• Vasodilators like calcium channel blockers or CCB and alpha-adrenergic blockers like Prazosin are prescribed in severe cases of acrocyanosis. Reasonable improvement is observed with Nifedipine and Diltiazem in some patients.

• The topical application of nicotinic acid derivatives and minoxidil is useful.

• Secondary acrocyanosis requires specific treatments for the underlying disease.

• A combination of Pentoxifylline and Diltiazem shows promising results in the remittent necrotizing type of acrocyanosis.

Conclusion

Acrocyanosis is a benign condition. Treatment is done for the underlying etiology of secondary acrocyanosis. The prognosis for acrocyanosis is good without the risk of morbidity. The patient can lead an everyday life. Early diagnosis and lifestyle modification prevent its morbidity.