In individuals with erythromelalgia, associated symptoms are typically relieved with cooling. More specifically, in almost all cases, affected individuals may experience pain relief by immersing the affected regions in ice water. However, according to experts, it is essential to note that the repeated immersion sometimes performed by those with severe erythromelalgia may actually serve to trigger symptom episodes (i.e., reactive flaring) and may lead to skin injury and potentially serious complications. Such complications may include infection; nonhealing skin sores (ulcers); softening and breaking down of skin due to abnormally prolonged exposure to moisture (maceration); and/or localized tissue loss (necrosis).
Many with the disorder may also experience symptom relief by exposing affected areas to cold air, such as through the use of air conditioners or fans, although again, excessive blowing air on the skin can cause its own cycle of problems (the equivalent of ‘windburn’). In addition, even those with mild disease may find themselves avoiding warm or hot temperatures in an effort to help minimize symptoms.
Many affected individuals find that symptoms worsen with a dependent (or “hanging down”) position. Accordingly, episodes may potentially be avoided or reduced by elevating involved regions.
Unfortunately, in some cases, the use of such measures as described above–such as avoidance of warm temperatures, ongoing elevation required by some with severe erythromelalgia, etc.–may significantly affect daily functioning.
For many patients, medications are available that can help to reduce symptoms.
Topical medications may go a long way towards helping with symptoms. The use of lidocaine topically such as in a lidocaine patch, and topical preparations designed to block the opening of sodium channels in nerve (amitriptyline combined with ketamine for example) have been described to be helpful in many patients, either alone or in combination with oral treatments.
Oral medications include calcium antagonists, magnesium selective serotonin reuptake inhibitors, tricyclic antidepressants, gabapentin or carbamazepine, antihistamines, clonazepam, misoprostol, cyproheptadine, and others. No single medication works for all EM patients, and some trial and error may be necessary. Some individuals with EM require lower doses of these drugs, and when started at higher doses, side effects can occur. Sometimes a combination of medications is more effective than one drug alone. Experts indicate that through such measures and careful ongoing monitoring, many affected individuals may obtain significant benefit.
Some patients with erythromelalgia develop the equivalent of a chronic pain syndrome, and this aspect should be intensively managed. In patients whose lives are severely impacted by the erythromelalgia, consideration should be given to engagement in a pain rehabilitation program, so that patients can learn techniques to live a more normal life despite the chronic pain of the erythromelalgia.
Genetic counseling may be of benefit for people with erythromelalgia and their families. Other treatment for the condition is symptomatic and supportive.