What is Dercum disease?
Dercum disease – also known as Adiposis Dolorosa or Anders’ syndrome and is a rare condition that is characterized by multiple, painful fatty lipomas (benign, fatty tumors) that occur chiefly in women of middle age. 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 and leg 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 with time. It causes problems with mobility in later stages.
What are the symptoms of Dercum disease?
This syndrome consists of four cardinal symptoms: (1) multiple, painful, fatty masses; (2) generalized obesity, or another fatty tissue disorder, such as lipedema (3) weakness and fatigability; and (4) mental disturbances, including emotional instability, depression, anxiety, and epilepsy.
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 other connective tissue diseases, such as Ethers Danlos, 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). In addition, other fat disorders such as lipedema are commonly associated with Dercums, as well as vein disease.
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, vein compression, and lymphatic channel compression and result in weakness swelling, pain, and skin changes.
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, or reliably predictive.
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. It is a very rare disease, and many clinicians are unaware of its existence. It is imperative that your clinician be experienced in the treatment of this rare disease.
Depending on the person, options can include weight reduction, surgery for painful lipomas (microcannula liposuction) and medications to control pain.
Surgical options include traditional excision vs microcannula liposuction. Surgical excision is much more invasive, and can be disfiguring in cases that are extensive. We favor microcannula liposuction as the first line treatment in severely symptomatic Dercums disease due to its significantly less invasive component, as well as excellent clinical results in our hands.
The downside to microcannula liposuction in comparison to traditional surgery is the lack of a clinical specimen to be evaluated by a pathologist, which for most patients, is significantly outweighed by its benefits over traditional surgery.
In general, because the fatty tumors in Dercums disease will recur to some degree, the major goal is pain reduction, mass reduction, and control of the disease.
In most cases we can obtain a significant clinical and functional benefit with minimal adverse risks or effect in the large majority of patients.