Erythromelalgia is a rare condition in which a patient has attacks of burning pain, warmth, redness and swelling in the feet and sometimes hands. The disease may result from an abnormality in the blood flow to the limbs. The condition occurs in isolation but also as a result of an underlying medical problem. In addition to physical, the disease also causes mental problems. A supportive program is helpful to manage symptoms. This consists of cooling the affected areas, medication and other therapies.

• Synonyms of erythromelalgia
• Epidemiology
• Causes
• Species
• Symptoms of feet (or hands)
• Diagnosis and examinations
• Treating attacks of burning pain, redness, heat and swelling

• Self-care
• Professional medical care

• Prognosis of disease
• Complications of condition

Synonyms of erythromelalgia

Well-known synonyms of erythromelalgia are:

• acromelalgia
• erythermalgia
• erythermalgia
• Gerhardt’s disease
• Mitchell’s disease
• Weir-Mitchell disease

Epidemiology

Erythromelalgia is a rare condition with an annual incidence of 0.25-1.3 per 100,000 patients. Women are affected more often than men. The symptoms most often appear in middle-aged patients, but in principle they appear at any age, including children.

Causes

An increased number of platelets in the blood (thrombocythemia) causes abnormalities in the arterioles (small arteries), resulting in abnormal blood flow to the limbs.

Species

Erythromelalgia is an isolated primary condition or secondary to several underlying conditions.Primary form Primary erythromelalgia occurs sporadically as a result of a new genetic change (de novo mutation) or is familial (hereditary) with an autosomal dominant inheritance pattern.Secondary form Sometimes the condition is secondary to various underlying bone marrow disorders involving abnormally increased production of certain blood cells (myeloproliferative disorders). Furthermore, many neuropathies (nerve disorders) also occur, but it is not clear to scientists whether this is a cause or a consequence of the disease. Many other conditions are already present in the patient. Huidvelden.nl has compiled a list of these. The disease may also appear due to the administration of certain drugs (such as Bromocriptine, nifedipine, nicardipine).Swollen, red feet are typical / Source: By Herbert L. Fred, MD and Hendrik A. van Dijk, Wikimedia Commons (CC BY-2.0)

Symptoms of feet (or hands)

The symptoms present on both sides of the body (bilateral), although sometimes one side is also affected (unilateral). The feet and sometimes the hands are particularly affected by the disease. The symptoms present episodic (in attacks) in most patients. However, the disease course and clinical picture are highly variable. For example, some patients experience sudden (acute) symptoms that spread, causing the symptoms to become very severe after a few weeks. However, most patients experience gradual (insidious) symptoms where the condition may be relatively mild for years.The patient has intense, burning pain in the feet and sometimes the hands (burning feet and burning hands). In addition, severe redness (erythema) occurs during the attacks in combination with an increased skin temperature (warmth) and swelling. During an episode, some affected patients also experience tingling pain or other symptoms associated with peripheral neuropathy (damage to peripheral nerves with pain, weakness, numbness and tingling especially in the hands and feet). Erythromelalgia often starts with occasional symptoms (for example, once a week or once a month), but it is possible for the episodes to increase in number and frequency. The attacks last from a few minutes to several hours. In rare cases, the symptoms spread from the feet to the legs and from the hands to the arms. Most patients have attacks late in the day, even at night in bed, causing sleep problems. Exposure to heat and physical exertion worsen the complaints, while they are reduced by cool conditions.

Diagnosis and examinations

A blood test is sometimes necessary / Source: Frolicsomepl, PixabayPhysical and diagnostic examination The patient receives a physical examination, but for making the diagnosis it is even more useful if the patient takes photos of the appearance of the feet (and/or hands) during an attack. Immersing the affected feet in warm water for ten to twenty also confirms the diagnosis. Going through the medical and family history is helpful. Sometimes further tests (such as blood tests and sometimes imaging tests) are needed to detect other underlying problems. Or else to rule out certain conditions with similar symptoms.Differential diagnosis The symptoms of the following diseases have a similar clinical picture to that of erythromelalgia and are therefore a differential diagnosis for the disease:

• frostbite
• cellulitis (skin infection with red and swollen skin)
• conversion
• Fabry disease (storage disease with fat build-up)
• Raynaud’s disease / Raynaud’s phenomenon (changes in skin color with cold and pain)
• a side effect of medicines
• essential thrombocythemia (thrombocytosis)
• googlochondria
• the complex regional pain syndrome (pain condition of limbs)
• hypercoagulability (thicker blood)
• hyperviscosity of the blood
• occlusive vasculopathies
• peripheral neuropathy
• polycythemia vera (blood disease with increase in all blood cells)
• reflex dystrophy
• systemic lupus erythematosus
• vasculitis (inflammation of a blood vessel)

Treating attacks of burning pain, redness, heat and swelling

Self-care

Immersing the affected areas in ice water and exposure to cold air (air conditioner, fan) often solves the problem. However, repeated immersion by patients with severe erythromelalgia sometimes leads to an activation of the attacks, which in these patients also leads to skin injuries and/or serious complications such as infection, non-healing skin ulcers (slow wound healing), maceration (softening and breakdown). of the skin due to abnormally prolonged exposure to moisture) and/or necrosis (localized tissue loss). Excessive exposure to blowing air may also lead to complications and the patient should avoid this. In addition, patients should avoid heat or warm temperatures. The symptoms of the affected areas may worsen when the areas are in a drooping position. The patient should therefore elevate the involved feet or arms. However, these measures are not equally effective for all patients.

Professional medical care

For many patients, medications are available that reduce symptoms. Applying the drug lidocaine topically (on the skin) is useful, possibly in combination with oral (taken by mouth) treatments of other medications. For this purpose, the doctor uses, for example:

• antihistamines (medicines to treat an allergic reaction)
• calcium channel blockers (medication for cardiovascular diseases)
• clonazepam
• cyproheptadine
• gabapentin or carbamazepine
• magnesium selective serotonin reuptake inhibitors
• misoprostol
• tricyclic antidepressants
• …

No single medication works for all erythromelalgia patients, so sometimes the doctor has to experiment with prescribing (combinations of) medications. The dosage is also different for all patients and side effects may also occur. Most patients take a combination of drugs to treat the condition. Continuous follow-up is necessary for these patients.

Prognosis of disease

Some patients with erythromelalgia develop the equivalent of a chronic pain syndrome, which requires intensive treatment and monitoring. In addition, the disease affects the quality of life, both physically and mentally. A pain rehabilitation program with psychotherapy is recommended for many patients.

Complications of condition

In addition to the pain and reduced quality of life, other complications may arise, such as:

• hypothermia (hypothermia due to prolonged and constant cooling of the affected body areas)
• necrosis and gangrene (tissue death with changes to the skin)
• ulcer formation

Necrosis or skin ulcers on the fingers and/or toes resulting in a secondary infection may lead to amputation.

read more

• Burning feet: Causes of burning, hot feet
• Burning sensations: Causes and locations of burning sensation
• Redness of skin: Causes of red rashes
• Foot pain: Causes of sore foot or foot pain