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.

History

Dercum's Disease was originally described in the medical literature in 1892 by the American doctor Frances Xavier Dercum.

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

Magnetic resonance imaging

Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...

 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
  Liposuction
  Liposuction, also known as lipoplasty , liposculpture suction lipectomy or simply lipo is a cosmetic surgery operation that removes fat from many different sites on the human body...
  
  ) has been used in some cases. It may temporarily relieve symptoms although recurrences often develop.

Medication

• Fentanyl
• Cannabis
  Cannabis
  Cannabis is a genus of flowering plants that includes three putative species, Cannabis sativa, Cannabis indica, and Cannabis ruderalis. These three taxa are indigenous to Central Asia, and South Asia. Cannabis has long been used for fibre , for seed and seed oils, for medicinal purposes, and as a...
  
  
• Lyrica
• The use of infliximab
  Infliximab
  Infliximab is a monoclonal antibody against tumour necrosis factor alpha . It is used to treat autoimmune diseases. Remicade is marketed by Janssen Biotech, Inc...
  
   and methotrexate
  Methotrexate
  Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
  
   has been proposed.
• Intravenous infusions of the local anaesthetic drug lidocaine
  Lidocaine
  Lidocaine , Xylocaine, or lignocaine is a common local anesthetic and antiarrhythmic drug. Lidocaine is used topically to relieve itching, burning and pain from skin inflammations, injected as a dental anesthetic or as a local anesthetic for minor surgery.- History :Lidocaine, the first amino...
  
   may give temporary relief from pain. Additional treatments of lidocaine may be necessary periodically to sustain the effect.
• Another analgesic drug, peroral mexiletine
  Mexiletine
  Mexiletine belongs to the Class IB anti-arrhythmic group of medicines. It is used to treat arrhythmias within the heart, or seriously irregular heartbeats. It slows conduction in the heart and makes the heart tissue less sensitive. Dizziness, heartburn, nausea, nervousness, trembling, unsteadiness...
  
  , may also be effective in eliminating pain for variable periods of time.

External links

• NORD (National Organization of Rare Disorders)
• Dercum's Disease (www.dercum.org)