Erythromelalgia Disorder Affecting Skin of Hand and Feet

med.jhmi. edu

Erythromelalgia is a rare disorder which affects the skin of the feet or hands or the arms, legs, ears and face as well, causing visible redness with intense heat and burning sensation. The term erythromelalgia describes the syndrome erythros – redness, melo as extremity and algia as pain. Its alternative name is erythermalgia which stresses the thermos or heat that is the essential factor of the syndrome.

The symptoms could start at any age and some people with this ailment could have had it from young age while in some it could surface in their later years of adulthood. It usually affects both sides of the body though it could affect just one side.

The pain and burning could tend to be extremely severe and people with this disorder usually make a lot of adjustment in their lifestyle to reduce the pain and burning sensation. In less severe cases, normal function like standing, walking, working exercising, sleeping and socializing could be affected.

Cooling the hot areas of the body could relieve the flare ups as well as elevating the affected areas could be of much help. Besides this, the use of ice and water soaking should be avoided as ice and water submersion could damage the skin.

Flare-ups of Severe Pain

People suffering from erythromelalgia suffer from flare ups of severe pain which could last for a few minutes to several days. These flare ups begin with an itching sensation that tends to worsen to extreme burning pain with tender mottled reddish skin.

The pain could be so severe that the person may tend to have difficulty to walk, stand, or perform the normal activities which could affect their work or social life. Symptoms of this disorder usually get triggered due to an increase in body temperature.

Besides this it could also trigger off after exercising, wearing gloves, socks or tight footwear, entering a warm room, being dehydrated or on consumption of alcohol or spicy food. The cause of erythromelalgia in most cases in not known though in some cases, an underlying cause could be found, either a medical condition or it could be a faulty gene.

Causes of Erythromelalgia

Erythromelalgia could be the outcome of underlying disease like having abnormal high levels of blood cells. It could either be due to many platelets in the blood or too many red blood cells, damage to the peripheral nervous system which is the network of nerves placed on the outer area of the brain and spinal cord where the damage is called peripheral neuropathy, MS or multiple sclerosis which is a disease of the nerves in the brain and the spinal cord.

In some cases of erythromelalgia, the disorder is the result of the change in a gene which may occur spontaneously or it could have been inherited from the parent indicating that it runs in the family. Other causes could be an autoimmune problem like lupus or rheumatoid arthritis wherein the immune system tends to attack the body’s own tissues.

The genes in the body contain genetic information or instructions to proteins in the body and genetic mutation takes place when the DNA changes thereby altering the genetic instruction resulting in erythromelalgia.

Different Treatment Based on Condition

Other causes could be due to changes in the SCN9A gene which provides information for sodium channel in pain nerves and changes in these channels could result in opening which could be easy and remains open longer than usual thus increasing or amplifying the pain.

The condition of hands and feet being affected the most is not identified. Various medication have given much relieve to the symptoms experienced by the patient though no specific drug has helped and different treatment needs to be experimented under the supervision of physicians together with the combination of different medication.

The physician could then discuss the various options best suited to the person. Some treatments would also need referral to specialist centre in order to check the benefits and potential side effects.

Other Pain Relieving Drugs/Intravenous Infusion – Reduce Pain 

There are other drugs of nerve pain that could be used to reduce symptoms which includes anti-epilepsy drugs or low doses of tricyclic anti-depressants which could be effective in pain relieve that are due to increased sensitivity or damage to the nervous system.

Moreover, drugs used for high blood pressure or Raynaud’s disease could also help the blood vessels to widen and prove to be beneficial in certain cases of erythromelalgia. In some case of this disorder, when the pain has not been relieved by medication taken by mouth or orally, another option through intravenous infusion could be used wherein the medicine is given directly in the bloodstream through a drip.

A local anaesthetic drug is also given to block sodium channels which could help the nerve related pain and is administered depending on the condition of the patient, the procedure of which is explained by the physician to the person suffering from erythromelalgia.