Dercum?s disease

Related Terms

Adiposa dolorosa, adiposis dolorosa, Anders' syndrome, Dercum's syndrome, fat, lipoma, lipomatosis dolorosa, liposuction, neurolipomatosis, pain.

Background

Dercum's disease is a very rare condition characterized by painful fatty lumps and nodules, called lipomas. Pain may be caused by the lipomas pressing on nearby nerves or by insufficient blood supply to the area and may be extreme and intense, continuous (lasting for hours), or may come and go in spasms. Patients may also experience more diffuse pain throughout fatty tissue. The condition is progressive and becomes worse over time.
Symptoms are usually first seen in adults, and the disease is less common in children and teenagers. The disease is most common in overweight postmenopausal women. However some patients who develop the disease are not obese. In general, Dercum's disease is thought to be a very rare condition.
The cause of Dercum's disease is not clear. Some evidence suggests it may be an inherited condition, but many cases have been reported that do not seem to involve genetic transmission from a parent to a child.
Currently, there is no known cure for Dercum's disease. Treatment focuses on management of symptoms, especially reduction of pain.

Signs and symptoms

General: Dercum's disease is a very rare condition characterized by painful fatty lumps and nodules, called lipomas. The condition is progressive and becomes worse over time.
The painful fat deposits may appear almost anywhere on a patient's body. Very often, they are found on the hips, the inside of the knees, the upper arms, the buttocks, the armpit, or the stomach. Rarely, they are found on the face or hands.
Pain: The pain that patients with Dercum's disease experience may be caused by the lipomas pressing on nearby nerves or by insufficient blood supply to the area and may be extreme and intense, continuous (lasting for hours), or may come and go in spasms. Pain usually increases when a lipoma is bumped or pressed. In some cases, just clothing touching the skin is enough to cause pain. Due to this pain, patients with Dercum's disease may have difficulty performing everyday tasks, such as walking, driving, or lifting objects.
Fatigue: Patients with Dercum's disease often experience fatigue and a loss of strength.
Mental problems: Some mild mental disturbances, including depression, confusion, and memory problems have been reported by patients with Dercum's disease. Some patients may have headaches or difficulty sleeping.

Diagnosis

Dercum's disease is very rare and difficult to diagnose, and there are no tests that can be used to conclusively diagnose the condition.
Some patients may find that Dercum's disease is not well known or accepted in the medical field. In some instances, doctors have assumed that the symptoms are imaginary.
Typically, a diagnosis of Dercum's disease may be made when a patient experiences at least three months of chronic pain in fatty tissue.

Complications

Swelling: For unclear reasons, patients with Dercum's disease may experience swelling of the skin, which can cause the skin to become loose and inelastic.
Numbness: In some instances, patients may experience numbness or a burning sensation in a limb, especially the hands or feet. This is usually caused by a lipoma that is pressing against a nerve.
Impaired heart/lung function: Lipomas may move to the heart or to the lungs and interfere with cardiac or pulmonary function. Although this occurs very rarely, it may lead to a potentially life-threatening situation.

Treatment

General: Currently, there is no known cure for Dercum's disease. Treatment focuses on management of symptoms, especially reduction of pain.
Drugs: Many drugs commonly used to relieve pain (such as analgesics like NSAIDs or acetaminophen) do not appear to be effective in treating Dercum's disease.
Lidocaine: One drug that has been used to treat Dercum's disease effectively is lidocaine, which is commonly used as an anesthetic. Patients receiving lidocaine intravenously over a 30-90 minute period have reported relief from pain lasting several weeks. Additionally, lidocaine has been administered using a patch. Side effects of lidocaine may include a drop in blood pressure, abnormal heart rhythms, dizziness, or headache.
Surgery: Surgical removal of lipomas usually is effective in reducing pain for a patient. However, surgery is not considered a cure, as lipomas often grow back after being removed.
Liposuction: Liposuction (a surgical procedure used to remove fat from the body) may be used to treat Dercum's disease. Removal of fat often leads to a reduction in pain in patients and an improved quality of life.

Integrative therapies

Note: Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease.
Traditional or theoretical uses lacking sufficient evidence:
Integrative therapies which lack sufficient clinical evidence for use in temporary pain relief from Dercum's disease symptoms include: acupuncture, massage, and hydrotherapy.

Prevention

High quality scientific evidence regarding prevention of Dercum's disease is currently lacking.

Author information

This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography

Amine B, Leguilchard F, Benhamou CL. Dercum's disease (adiposis dolorosa): a new case-report. Joint Bone Spine. 2004 Mar;71(2):147-9.
Campen R, Mankin H, Louis DN, et al. Familial occurrence of adiposis dolorosa. J Am Acad Dermatol. 2001 Jan;44(1):132-6.
DeFranzo AJ, Hall JH Jr, Herring SM. Adiposis dolorosa (Dercum's disease): liposuction as an effective form of treatment. Plast Reconstr Surg. 1990 Feb;85(2):289-92.
Devillers AC, Oranje AP. Treatment of pain in adiposis dolorosa (Dercum's disease) with intravenous lidocaine: a case report with a 10-year follow-up. Clin Exp Dermatol. 1999 May;24(3):240-1.
National Human Genome Research Institute. .
Natural Standard: The Authority on Integrative Medicine. .
Wortham NC, Tomlinson IP. Dercum's disease. Skinmed. 2005 May-Jun;4(3):157-62.

Causes

The cause of Dercum's disease is not clear. Some evidence suggests that it may be due to inheritance of a defective gene, but in many cases the disease appears to occur randomly. No gene or mutation that causes Dercum's disease has been identified.

Risk factors

Postmenopausal overweight women appear to have the greatest risk for developing Dercum's disease. Only about one in 20 individuals with Dercum's disease is male. The condition has also been observed in teenagers and children, but it is not as common.