Dercum's Disease
Adiposis dolorosa, also known as Dercum's Disease is a rare condition characterized by multiple, painful lipomas. These lipomas mainly occur on the trunk, the upper arms and upper legs. The diagnosis of Dercum's disease implies a long, chronic pain syndrome of debilitating nature. The exact cause of Dercum’s disease is unknown.
Symptoms
Multiple painful lipomas are the cardinal symptom of this disease. The pain can be very intense and can be described as aching, stabbing, smarting or burning. The pain is chronic and progressive, but varies much in cycles. Other common symptoms are:
- Various areas of the body may swell for no apparent reason. The fingers becomes clumsy, a person may drop things and sometimes the fingers go numb.
- Disturbed sleep, many have difficulties going to sleep because of the pain, but other forms of sleeping disorders also occur.
- General fatigue, worsening with even mild activity.
- Tendency to become black and blue; this seems to arise spontaneously or after alleviated blow.
- Stiffness after resting especially in the mornings.
- Skeletal pain in wrist, elbows, hips, tail bone and the long bones of the arms and legs.
- Headache, usually a combination between tension headache and classic migraine.
- Memory lapses and concentration difficulties making it difficult to learn new things and to accomplish intellectually demanding jobs.
- Feeling hot is often felt by the patients, some have 37.5 to 39 Celsius degree fever several weeks in a row, with increased pain and incapacity to work as a consequence.
- Tenderness under the feet, akin to walking on glass.
- Tenderness in the skin, difficulties in wearing tight fitting clothes or taking a shower.
- Infection sensitivity. Frequently increased pain during infections or active allergy attacks.
Causes
The cause of the disease is unknown and poorly researched. The symptoms and the lack of treatments can seriously affect the quality of life.
Diagnosis
A diagnosis of Dercum's disease is based on patient history and the physical findings. There are no specific laboratory test for this disease. Since the disorder does not show on the outside it can be hard for others to see the level of difficulties and discomfort the patient experiences. More than half of the patients are unable to work. The knowledge about the disorder is not well known and many patients are poorly treated. Some lab tests are currently being used to aid in the diagnosis but are not diagnostic themselves. Ultrasound and MRI are also useful in seeing the nodules found in visceral fat. Magnetic resonance imaging can play a role in diagnosis.
Treatment
Treatment of Dercum's disease is usually targeted towards pain relief rather than removal. Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease. There are not enough studies done to prove that diet and supplements could help with the disease.
Treatment methods include:
Surgery
Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases. It may temporarily relieve symptoms although recurrences often develop.
Gluten-free diet
Dercum's disease is primarily an autoimmune disease triggered to a long term intolerance for gluten. A gluten-free diet reduces the inflammation after an extended period of time (1 to 3 months).
Stress Reduction
Pain and discomfort coming from the lipoma and fatty tissues are triggered with high levels of cortisol. First step in the relieve for dercum's disease is removal of the most painful lipoma (surgery, best done by a cosmetic surgeon due to the many incisions that have to be made) followed by steps to lowering cortisol levels in the blood (stress reduction). Avoiding contact with cold air and temperatures on legs, arms and body contributes to calmer tissue.
Medication
- Fentanyl
- Lyrica
- The use of infliximab and methotrexate has been proposed.
- Intravenous infusions of the local anaesthetic drug lidocaine may give temporary relief from pain. Additional treatments of lidocaine may be necessary periodically to sustain the effect.
- Another analgesic drug, peroral mexiletine, may also be effective in eliminating pain for variable periods of time.