Adiposis dolorosa

Adiposis dolorosa, also known as Dercum's Disease^[1] is a rare condition characterized by multiple, painful lipomas. These lipomas mainly occur on the trunk, the upper arms and upper legs. The understanding of the cause and mechanism of Dercum disease remains unknown. The lipomas cause nerve compression and result in weakness and pain.^[2] The diagnosis of Dercum's disease implies a long, chronic pain syndrome of debilitating nature.

History

Dercum's Disease was originally described in the medical literature in 1892 by the American doctor Frances Xavier Dercum.^[3]

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:^[4]

• 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.

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. Magnetic resonance imaging can play a role in diagnosis.^[5]

Treatment

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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. ^[6] There are not enough studies done to prove that diet and supplements could help with the disease.^[7]

Treatment methods include:

Surgery

• Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases.^[8] It may temporarily relieve symptoms although recurrences often develop.

Medication

• Ziconotide
• Medical cannabis
• Fentanyl
• Lyrica
• The use of infliximab and methotrexate has been proposed.^[9]
• Intravenous infusions of the local anaesthetic drug lidocaine may give temporary relief from pain.^[10] 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.^[11]

References

External links

• NORD (National Organization of Rare Disorders)
• Dercum's Disease (www.dercum.org)
• Clip from Mystery Diagnosis about Dercum's Disease