Dercum

What is Dercum’s Disease

• Dercum's disease is an extremely rare disorder characterized by multiple, painful growths consisting of fatty tissue (lipomas).
• These growths mainly occur on the trunk, the upper arms and upper legs and are found just below the skin subcutaneously.
• Pain associated with Dercum's disease can often be severe.
• Pain may be caused by these growths pressing on nearby nerves.
• Dercum's disease mainly occurs in adults and more women than men. In some cases, individuals may also experience weight gain, depression, lethargy, and/or confusion.
• The exact cause of Dercum's disease is unknown.

Synonyms of Dercum's Disease

• Adiposis Dolorosa
• Fatty Tissue Rheumatism
• Juxta-Articular Adiposis Dolorosa
• Lipomatosis Dolorosa Morbus Dercum's

Symptoms

• The characteristic finding of Dercum's disease is the slow formation of multiple, painful growths consisting of fatty tissue (lipomas) that are found just below the surface of the skin.
• Pain may vary from mild discomfort when a growth is pressed or touched to severe pain that is disproportionate to the physical findings.
• Pain can last for hours and may come and go or last continuously.
• In severe cases, pain may worsen with movement. The exact reason for pain associated with Dercum's disease is unknown, but may occur because the lipomas press on nearby nerves.
• Lipomas may be found in any part of the body, although they are rare in the head and neck.
• The trunk, upper arms and upper legs are most often affected.
• Some individuals with Dercum's may experience swelling of various areas of the body, especially the hands.
• Swelling occurs for no apparent reason and often disappears without treatment.
• Significant weight gain is a common occurrence for most individuals affected by Dercum's disease.

Additional Symptoms

• Fatigue
• Generalized weakness
• A tendency to bruise easily.
• Headaches
• Irritability
• Stiffness after resting, especially in the morning.
• An association with bouts of depression, memory or concentration problems.
• Susceptibility to infection has been noted in some cases.
• Arthritis.
• High blood pressure (hypertension).
• Congestive heart failure
• Sleep disorders
• Dry eyes
• Myxedema (a condition due to an underactive thyroid that is characterized by dry skin, swelling around the lips and nose, and mental deterioration.)

Causes

• The exact cause of Dercum's disease is unknown. In most cases, Dercum's disease appears to occur spontaneously for no apparent reason (sporadic).
• Some have suggested that Dercum's disease may be an autoimmune disorder- a disorder in which the body's immune system mistakenly attacks healthy tissue.
• Disturbances in endocrine function and improper breakdown (metabolism) of fat have also been proposed as potentially playing a role in the development of this disorder.

Standard Therapies

Diagnosis

• A diagnosis of Dercum's disease is suspected based on a detailed patient history, a thorough clinical evaluation and identification of characteristic multiple fatty growths.
• Surgical removal and microscopic study (biopsy) of affect tissue confirms that these growths are lipomas.

Treatment

• Specific treatment exists for Dercum's disease. Treatment is directed toward the specific symptoms that are apparent in each individual and is primarily focus on easing the characteristic painful episodes.
• Various painkillers (analgesics) have been tried with limited effectiveness. Injections of corticosteroids have also been used to treat individuals with Dercum's disease.
• Surgical excision of fatty tissue deposits around joints may temporarily relieve symptoms although recurrences often develop.
• Liposuction has been used as a supportive treatment for some individuals with Dercum's disease and may provide an initial reduction in pain and improvement in quality of life.
• Psychotherapy and consultation with pain management specialists may be helpful for enabling affected individuals to cope with long-term intense pain.

Affected Populations

Dercum's disease affects females more often than males with some reports citing the disease is as 20 times more common in women.

• Dercum's disease can affect individuals of any age.
• The majority of cases are women between the ages of 45-60, especially overweight, postmenopausal women.
• Although an extremely rare occurrence, it has been reported in children.

Source: Ms Sruthi, Volunteer