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Learning About Dercum Disease
What is Dercum disease?
Dercum disease - also known as Adiposis Dolorosa, Anders’ syndrome and
Dercum-Vitaut syndrome - is a rare condition that is characterized by multiple,
painful fatty lipomas (benign, fatty tumors) that occur chiefly in post-menopausal,
obese women of middle age. However, although it is 20 times more common in women, 16 percent of the reported cases are males and it can also occur in people who are not obese
The lipomas are located primarily on the trunk region and on the extremities
close to the trunk. Unlike ordinary lipomas, there is also pain that can be
severe and sometimes debilitating. Dercum disease is a chronic condition, meaning
that it is a long lasting condition. In addition, it tends to be progressive.
What are the symptoms of Dercum disease?
This syndrome consists of four cardinal symptoms: (1) multiple, painful, fatty
masses; (2) generalized obesity, usually in menopausal age; (3) weakness and fatigability;
and (4) mental disturbances, including emotional instability, depression, epilepsy,
confusion and dementia.
The pain can last for hours, can be paroxysmal (occurring only at certain times)
or continuous, and worsens with movement. Dercum disease is often associated
with generalized weakness, depression, and irritability. The condition can also
be associated with early congestive heart failure, myxedema (a condition associated
with severe hypothyroidism), joint pain, paroxysmal flushing episodes, tremors,
cyanosis (bluish discoloration of the skin), hypertension (high blood pressure),
headaches, and epistaxis (nosebleeds).
View an image of a lipoma: Lipoma
- arm
From MEDLINEplus, the National Library of Medicine
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.
NHGRI Clinical Research on Dercum Disease:
Currently, NHGRI is not conducting research on Dercum Disease
Additional Resources for Dercum Disease
The information on this page was adapted from the following resources:
- eMedicine: Adiposis Dolorosa, Marjan Yousefi, M.D., Department of Dermatology, Geisinger Medical Center
- NORD Guide to Rare Disorders: "Adiposis Dolorosa," Rebecca B. Campen, M.D.
(2003)
Last Updated: February 25, 2009
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