Q: My husband has been diagnosed with erythromelalgia of the hands, although his pain isn’t triggered by heat but by cold weather. He is very rarely without severe pain and burning symptoms. Often times his hands are manageable but at others, they are not. He takes an aspirin every day. Hid physician has tried other medications but nothing has worked. Some of his symptoms are contrary to his diagnosis.
Is there any other condition that could manifest itself in this same manner? This is a very debilitating disease.
A: Erythromelalgia (EM), once known as acromelalgia, red neuralgia, erythermalgia and Mitchell disease is a rare disorder characterized by periods of burning pain, redness and warmth in the hands and feet. Blood vessels become blocked periodically, causing them to swell. The blockage is accompanied by bouts of severe pain that radiates in the soft tissue. Triggers may be from exertion, pressure, heat, insomnia, stress, and more.
EM may occur either as a primary or a secondary disorder. The primary type is associated with an issue in and of itself, or it may be a symptom of an underlying disorder. The secondary form may result from small fiber peripheral neuropathy or from other causes. EM may develop in individuals with mercury or mushroom poisoning, high cholesterol levels, specific autoimmune disorders, or in the presence of a normal platelet count in those with myeloproliferative disorder. Symptoms are often symmetric and affect the lower extremities more often than the upper ones. Primary form attacks vary from one extreme to another. They may last as short a period as one hour or as long as months at a time, may be infrequent or occur numerous times each day, present gradually taking years to be fully recognized or may be intense at onset. Raynaud’s phenomenon often co-exists in patients with EM.
EM is rare, occurring in 1.3% of every 100,000 individuals, with women being more prone than men according to recent research. About 5% of those with the condition have the primary inherited form of the disease.
The condition is difficult to diagnose and may take months since no specific testing is available for the condition. Once the primary form (not the secondary type) has been diagnosed, a plan of treatment can be initiated. Primary EM management focuses on treating painful symptoms only, and avoiding triggers that cause the attacks. Cold water baths or soaking the hands in cold water is heavily discouraged because the acts will precipitate problems, cause skin damage and ulceration. While unsupported medically and only anecdotal, there are medications patients have tried with some degree of success. Mild cases may respond to such medications as tramadol or amitriptyline, while more severe cases may necessitate opioids. Reduced capillary density has been seen microscopically during flareups, and reduced capillary perfusion is observed. When it comes to the secondary form, the best treatment method is to treat the underlying cause. Elevation of the extremities of the affected limbs is believed to lessen symptoms.
Because you admit some of your husband’s symptoms don’t fit what you’ve been told, I believe he should be under the care of a neurologist, simply because the paradoxical presentation suggests your diagnosis may be in question. You might ask him or her about Lyrica or other medications used to treat peripheral neuropathy as a possible treatment. Good luck.
