What is Weir-Mitchell Disease & How is it Treated?

What is Weir-Mitchell Disease?

Weir-Mitchell Disease which is also known by the name of erythromelalgia is an extremely rare pathological condition primarily affecting the lower extremities. The affected individual experiences intense burning and pain in the lower extremities and in some cases the upper extremities as well. The affected extremities also become intensely erythematous and warm. This is more or less continuous in nature.

Weir-Mitchell Disease normally affects both extremities at one time but in some cases unilateral symptoms have also been exhibited by the affected individual. The disease progression is extremely variable in Weir-Mitchell Disease and the severity of the symptoms differs on a case to case basis. It has also been seen the onset of the condition is also quite variable. In some cases the onset of symptoms is pretty gradual and insidious where the affected individual may have symptoms which are so mild that they might not even notice it for years, while in some cases the onset of symptoms are extremely acute and the symptoms of Weir-Mitchell Disease may become severe in a matter of days to weeks.

There is no known cause for Weir-Mitchell Disease, although some studies suggest that this condition may be caused by abnormalities in the blood vessels in which there is abnormal constriction and dilation of certain blood vessels causing abnormalities in the blood flow through these vessels.

These abnormalities normally occur in blood vessels which cater to the blood supply to the lower extremities although sometimes the blood vessels catering to the upper extremities may also get affected as a result of Weir-Mitchell Disease. Weir-Mitchell Disease may occur as a single entity or may occur as a result of certain underlying medical conditions. In some cases, a hereditary link has been noted in the development of Weir-Mitchell Disease.

What is Weir-Mitchell Disease?

What are the Causes of Weir-Mitchell Disease?

The root cause of Weir-Mitchell Disease is not yet known, but studies suggest that it is caused due to abnormalities in the constriction and dilation of certain blood vessels, especially those supplying blood to the lower extremities and sometimes the upper extremities which affect the blood flow to these regions causing the classic symptoms of Weir-Mitchell Disease.

Additionally, studies also suggest that one of the reasons for the development of Weir-Mitchell Disease is abnormal narrowing of the sphincters of the blood vessels and opening of the arteriovenous shunts. The function of these sphincters is to control blood flow from the small capillaries and arteries to the blood vessels.

When the blood flows through these arteries, they carry with them oxygen and other nutrients along with them. The arteriovenous shunts are blood vessels which connect the arteries and veins and function to regulate the temperature of the body. This abnormal narrowing of the sphincters of the blood vessels and opening of the arteriovenous shunts causes increased blood flow but decreased oxygen and nutrients in the affected skin areas causing a sensation of burning, pain, and erythema of the skin which are characteristic features of Weir-Mitchell Disease.

What are the Symptoms of Weir-Mitchell Disease?

The classic presenting feature of Weir-Mitchell Disease is pain, burning, erythema, and warmth in the lower extremities, especially the feet. There can also be swelling associated with it. In some cases, these features may also be seen in the upper extremities as well.

These symptoms are experienced bilaterally, although in some cases unilateral symptoms are also observed. Unilateral symptoms are mostly observed in cases where certain other medical conditions are responsible for the development of Weir-Mitchell Disease.

In some cases the onset of the symptoms are gradual and extremely mild such that the affected individual may not even notice the symptoms for many years whereas in some cases the symptom onset is extremely acute progressing rapidly in a matter of days to weeks to a severe level.

In majority of the cases, the symptoms of Weir-Mitchell Disease tend to get worse with time and the symptoms may start spreading from the lower extremities upwards to involve the face as well and from the upper extremities downwards. These symptoms tend to get worse with exposure to sunlight and are relieved when the individual goes to a cooler area. This is perhaps the symptom which is solely present in cases of Weir-Mitchell Disease.

How is Weir-Mitchell Disease Diagnosed?

The diagnosis of Weir-Mitchell Disease is basically made from the symptoms that the affected individual experiences along with a thorough clinical evaluation and tests. If the patient has a family history of Weir-Mitchell Disease then it also helps in pinpointing towards a diagnosis of this condition.

Specialized testing may be done to rule out other condition causing similar symptoms. The most clinching evidence of this condition is the worsening of the symptoms with exposure to sunlight and relief of symptoms when in cooler areas. This virtually confirms a diagnosis of Weir-Mitchell Disease.

How is Weir-Mitchell Disease Treated?

As stated above, relief of symptoms can be obtained with adequate cooling and thus if an affected individual with Weir-Mitchell Disease is subjected to gradual cooling then he or she may have relief of symptoms. This is usually done by putting the feet in ice cold water. This provides instant relief from symptoms of Weir-Mitchell Disease.

Although this form of treatment may relieve the symptoms but it may lead to certain undesirable complications like skin injuries, blisters, infections, and skin ulcers which may sometimes be nonhealing.

Tissue necrosis may also occur if the feet re-immersed in ice cold water repetitively for a long period of time. Many people with this disorder may also experience symptom relief by placing them in cooler environment like an air conditioned room.

Elevating the involved extremity is yet another way of relieving the symptoms of Weir-Mitchell Disease. These measures may be quite helpful in alleviating the symptoms of Weir-Mitchell Disease but on the hindsight such measures may significantly dent the daily functioning of the affected individual due to Weir-Mitchell Disease.

There are also certain medications that can help to alleviate the symptoms to certain degree of Weir-Mitchell Disease. Topical medications have also shown good benefit in alleviating the symptoms of Weir-Mitchell Disease.

The most preferred topical agent for relief of symptoms of this condition is lidocaine gel which masks the pain impulses and also relieves the symptoms of burning. Some of the oral medications that can be used to treat Weir-Mitchell Disease are calcium antagonists, antidepressants, antihistamines, and the like.

It should be noted here that an individual may not find that the medication he or she has been prescribed is effective right from day one. In some cases some medications may work while other may not. Thus, physicians may try a combination of a few medications until they identify the medication that bests suits the patient and helps them in relieving the symptoms of Weir-Mitchell Disease.

Some patients with Weir-Mitchell Disease may also benefit from a pain management program in which physicians make use of controlled substances in order to relieve pain so as to help the patients carry out their activity of daily living which becomes significantly impacted as result of Weir-Mitchell Disease.

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