Erythromelalgia

Erythromelalgia is a rare condition that can be quite devastating for its sufferers as well their families. Read on to know what is Erythromelalgia as well as its causes, symptoms, diagnosis and treatment.

Erythromelalgia Definition

Doctors and medical researchers define Erythromelalgia as a rare type of neurovascular peripheral disease that is characterized by pain and inflammation in the hands. The blood vessels in the hands suffer from episodes of obstruction followed by increased blood flow.

Picture 1 – Erythromelalgia

Erythromelalgia Synonyms

The condition is also known by other names like,

• Mitchell’s disease
• Acromelalgia
• Red neuralgia
• Erythermalgia

Erythromelalgia Types

The condition is differentiated into two types:

Primary Erythromelalgia

It results from genetic mutation and presents symptoms that are often triggered by heat, changes in temperature, spicy foods, alcohol and over-exertion or exercise.

Secondary Erythromelalgia

It can result from certain autoimmune disorders, essential thrombocytosis, mercury poisoning, mushroom poisoning and Hypercholesterolemia.

Erythromelalgia Symptoms

This condition is characterized by episodes of redness, inflammation and pain in different parts of the human body, especially the hands and feet. In some cases, the aches caused by this disorder can be so weakening as to hinder daily activities such as walking and wearing shoes. A person affected by this disorder may also be unable to attend school or office regularly.

The intensity of symptoms can widely vary from one patient to another. Some affected individuals experience moderate itchiness and tingling sensations which persist for only a few minutes. Others suffer from acute pain in their hands or feet. Some patients may also suffer from acute skin discoloration for several months or at least weeks at a time.

Generally, the discomforting symptoms are triggered by warm temperatures or friction due to constant skin contact with gloves and shoes. However, symptoms can also flare-up without any external stimulation. Very rarely, redness and burning symptoms have been found to affect other regions of the body, such as the ears and the scalp.

What Causes Erythromelalgia?

In most cases, the causes of Erythromelalgia are unclear and not identifiable. However, certain factors have been suspected as Erythromelalgia causes. These are:

Age

Progressing age is believed to cause as well as aggravate the symptoms of this disorder. This is because most patients suffering from Idiopathic Erythromelalgia are found to be above 60 years old.

Genetic conditions

Young sufferers may experience symptoms if any genetic blood vessel condition is found to be present in them. In rare circumstances, Erythromelalgia itself may run down generations in the same family. This usually happens when any of the parents of the suffering infant is a carrier of a particular genetic mutation responsible for the disease.

Autoimmune Disorders

Presence of an underlying autoimmune disease, such as diabetes or lupus, is also believed to be associated with the development of this condition.

Diseases

The condition is also suspected to arise due to the presence of other conditions like Sciatica, Myeloproliferative disease, Hypercholesterolemia, Mushroom poisoning, Fabry’s disease, Small fiber peripheral neuropathy and Mercury poisoning.

Neurological Problems

The disease is also supposed to occur due to the presence of neurological conditions, such as Multiple Sclerosis.

Medicinal side effects

The condition may also result due to side effects arising from use of ergot derivatives like Nifedipine, Verapamil Pergolide and Bromocriptine.

Erythromelalgia and Genetics

As aforesaid, some cases of Erythromelalgia are believed to arise from mutations in the SCN9A gene. These genetic mutations result in a NaV1.7 sodium channel that stays open for a greater duration than in usual cases. This results in an increase of sodium ion flow into nociceptors. This elevation in the flow of sodium ion increases the transmission of pain signals. It leads to the appearance of the signs and symptoms of Erythromelalgia.

Approximately 15% cases of this condition are thought to be a result of mutations in the SCN9A gene. Some cases are believed to occur due to a non-genetic factor or as a result of mutations in one or more unidentified genes.

Erythromelalgia Triggers

The discomforting episodes associated with this syndrome are generally triggered by an increase in body temperature. A rise in body heat may result from workouts, wearing tight socks or shoes for too long or staying in warm environs for a prolonged duration. Ingestion of spicy foods or alcohol may also give rise to an episode. With advancing age of sufferers and progression of the disease, there may be constant redness in the hands and feet which can extend to other areas such as the face and the upper legs.

Erythromelalgia Diagnosis

This condition is difficult to detect due to the lack of specific diagnostic tests. A dermatologist typically makes an initial diagnosis of the disorder based on the signs reported by a patient as well as observing the physical characteristics of his or her hands and legs. If the affected individual is not experiencing the symptoms during diagnosis, the doctor may try to induce them by soaking the extremities in hot water. They may also look for symptoms of any underlying condition with the aid of imaging scans, neurological exams and blood tests.

A proper diagnosis can take several months and patients may have to consult multiple specialists to find out their problem.

Erythromelalgia Differential Diagnosis

The Differential Diagnosis of Erythromelalgia consists of differentiating the disease from other disorders that produce similar symptoms. These include:

• Complex regional pain syndrome (CRPS)
• Small-fiber neuropathies
• Diabetes Mellitus

Erythromelalgia Treatment Options

Know about the treatment approach followed for two types of Erythromelalgia.

Primary Erythromelalgia Treatment

The management of Primary Erythromelalgia is symptomatic. The treatment of the condition involves addressing only the painful symptoms. Specific management tactics includes avoiding trigger factors responsible for the attacks, such as:

• Heat
• Changes in temperature
• Over exertion or exercise
• Alcohol
• Spicy foods

It is important to stay in a cool environment to keep the symptoms in control. Though it helps to maintain a cool body temperature, doctors discourage the use of cold water showers. It can result in damage or death of tissues (necrosis) in sufferers of extremely painful Erythromelalgia.

Secondary Erythromelalgia Treatment

For Secondary Erythromelalgia, primary treatment involves curing the underlying disorder. Use of aspirin may help decrease the symptoms of the condition. Often, it becomes necessary to manage the symptoms by lowering the temperature of the body. This can be achieved by elevating the limbs or placing them in cool water. The pain associated with the disease is acute and cured in an isolated manner. It is still unclear why the painful episodes related with Erythromelalgia mainly affect the extremities, i.e. the hands and feet.

Erythromelalgia Surgery

Treatment of Erythromelalgia depends on the severity of symptoms as well as the presence of any underlying medical condition. Generally, medicines and home remedies are enough to alleviate the problems. However, some patients finally require surgical cure to interrupt nerve connections and prevent acute complications.

If all other curative procedures fail to improve the condition, a surgical treatment may be considered. A process known as Sympathectomy includes surgical snipping of the nerve ends that transmit pain signals from the hands and feet. The process, though having serious risks associated with it, is usually regarded to be more beneficial when compared to the acute impacts of debilitating Erythromelalgia.

Erythromelalgia Natural Treatment

The natural cure of Erythromelalgia is said to be possible by consumption of foods rich in Vitamin B such as

• Whole grains
• Nutritional yeast
• Brown rice
• Peanuts
• Raw wheat germ
• Dairy products, including butter and milk
• Various yellow and green vegetables

These nutritious food items can help boost the immune system of sufferers as well as provide relief from the symptoms.

Erythromelalgia Relief

Home remedies and lifestyle modifications are usually enough to tackle this problem, particularly in sufferers of idiopathic Erythromelalgia. Doctors may advise patients to avoid activities like

• Wearing shoes
• Staying outdoors on hot days
• Intense workouts for long duration

During active flares, patients of this condition should also try immersing their hands and feet in cool water. In more serious cases, sufferers may be prescribed with a soothing topical cream. Oral anti-inflammatory drugs and Aspirin have been found to be effective in decreasing the intensity of symptoms in many patients.

The Erythromelalgia Association (TEA)

It is a non-profit organization based in Wethersfield that is dedicated to research and education associated to this condition. The institution has been founded with the aim to promote understanding of the conditions and symptoms of Erythromelalgia.

Erythromelalgia and Burning Feet Syndrome

Erythromelalgia is believed to give rise to a condition called “Burning Feet Syndrome”, that is common among individuals over 50 years of age. Burning Feet Syndrome is accompanied by discomforting signs like inflammation and sweating of foot.

Erythromelalgia Pictures

Here are some useful Erythromelalgia images that you may use for reference. Check out these Erythromelalgia photos to know all about the physical appearance of people affected by this condition.

Picture 2 – Erythromelalgia Picture

 

Picture 3 – Erythromelalgia Image

Living with this painful condition deteriorates the quality of life by restricting mobility and giving rise to the feelings of depression and social alienation. The condition, like many other rare disorders, may take years to get diagnosed and treated in an appropriate manner.