Rare Diseases : Dercum's Disease

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 are affected than men. In some cases, affected individuals may also experience weight gain, depression, lethargy, and/or confusion. The exact cause of Dercum’s disease is unknown.

Symptoms of Dercum syndrome


The list of signs and symptoms mentioned in various sources for Dercum syndrome includes the 8 symptoms listed below: 

• Painful skin lumps 
• Asthenia 
• Headache 
• Absence of menstruation 
• Bruises 
• Reduced sweating 
• Reduced skin sensitivity 
• Fatty skin lumps

What causes Dercum disease?

The understanding of the cause and mechanism of Dercum disease remains unknown. The origin of the pain is obscure, and the disease is better known as a description of its symptoms rather than as a physiologic or metabolic process. The fatty deposits (lipomas) cause nerve compression and result in weakness and pain.

Some cases reported in the literature have suggested possible causes for Dercum disease, such as the use of corticosteroids, a disturbance of endocrine function, or a genetic cause since it seems to run in some families. Some researchers have suggested that Dercum disease is an autoimmune disorder (a condition that occurs when the body's immune system attacks normal, healthy body tissue). However, no single cause has been pinpointed.

What do we know about heredity and Dercum disease?

Dercum disease has been reported to occur in families, and in some of these cases it seems to be inherited as an autosomal dominant trait (this means that one out of a person's two copies of the gene, inherited from one parent, is altered). However, most reported cases of this condition are sporadic (the only case to occur in the family).

What is the treatment for Dercum disease?

There is no "cure" for Dercum disease. At the present time, treatment for this condition is symptomatic, meaning that it focuses on one symptom at a time rather than the whole condition. Depending on the person, options can include weight reduction, surgery for the most painful lipomas, and medications to control pain. Liposuction has been used in some cases.

Sources : 
http://health.howstuffworks.com/diseases-conditions/rare/10-rare-diseases3.htm
http://www.rightdiagnosis.com/d/dercum_syndrome/symptoms.htm
http://www.genome.gov/17516629