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About Dercum's disease

Dercum's disease also referred to as Anders' syndrome, Adiposis Dolorosa, or Dercum-Vitaut syndrome is one among the rarest medical conditions. It is manifested with painful, multiple lipomas (benign tumor that develops from fat cells) that chiefly affect middle-aged, obese women during post-menopausal period. In general, it can also affect non-obese patients and around 20 percent of females are more prone to it. However, it is also presented by nearly 16 percent of males.

The condition is characterized with multiple lipomas that develop primarily over the trunk including extremities near the trunk region. These lipomas present with severe, debilitating pain unlike ordinary lipomas (benign tumors). In fact, Dercum's disease is a chronic illness (long lasting) with a progressive nature.

Define Dercum's disease

Dercums disease or Dercum disease is medically well-known as Adiposis Dolorosa and it is defined in different medical terminologies as follows:

According to Dorland's 27th edition text in the National Library of Medicine (IGM Metathesaurus), it is defined as "A disease accompanied by painful localized fatty swellings and by various nerve lesions. It is usually seen in women and may cause death from pulmonary complications."

According to the paraphrased version of the National Organization of Rare Diseases (NORD):

"Dercums Disease is a rare disorder in which there are fatty deposits which apply pressure to the nerves, resulting in weakness and pain. Various areas of the body may swell for no apparent reason. The swelling may disappear without treatment, leaving hardened tissue or pendulous skin folds."

What is Dercum's disease?

Dercum's disease is a rarest, severe disorder that presents with multiple, painful, fatty tissue over-growths referred to as lipomas (benign fatty tumors). These fatty growths typically develop on the upper arms, trunk, and upper part of legs usually underneath the skin surface (subcutaneously). Severe pain characterized with Dercum's disease is due to the pressure impingement on the surrounding nerves and blood vessels as a result of these developing lipomas. Dercum's disease mainly affects adults, especially women than men. Some of them present with other manifestations like depression, confusion, weight gain, and lethargy.

What are the other names for Dercum's disease?

The other names for Dercum's disease include dercum disease, Juxta-Articular Adiposis Dolorosa, Adiposis Dolorosa, Lipomatosis Dolorosa Morbus Dercum's, Fatty Tissue Rheumatism, Dercum-Vitaut syndrome and Anders' syndrome.

What causes Dercum's disease?

The exact possible cause of Dercum's disease is not yet known. In fact, the concept of major cause and mechanism of Dercum's disease is unknown. The pain associated with the condition is obscure, and can mostly be due to the compression of the nerve by the fatty growths (lipomas) leading to weakness.

Generally, Dercum disease is known to run in some families, and is considered as an autosomal dominant trait in some cases. However, majority of the cases are known develop this condition in sporadic form. According to the medical literature, certain possible causes that lead to the disorder include intake of corticosteroid medications, genetic mutation and a disrupted function of the endocrine system.

According to some researchers, it has been stated that Dercum's disease is an autoimmune disorder as the immune system attacks the normal tissues in the body however no supportive evidence has been presented yet. Some of the suggested, but unconfirmed causes of Dercum's disease are dysfunction of the adipose tissue, including nervous system; mechanical pressure on nerves; trauma.

What are the Signs and Symptoms of Dercum's disease?

Dercum's disease, usually known Adiposis dolorosa, is a rare, chronic condition where an affected subject presents with multiple, severely painful lipomas over the trunk, upper arms and upper part of lower extremities.

Generally, the syndrome typically presents with four cardinal symptoms such as:

(1) Multiple, fat tissue growths (lipomas) that cause severe pain

(2) Middle aged women at menopausal period present with generalized obesity

(3) Weakness and easy fatigue

(4) Mental disturbances like depression, emotional instability, confusion, epilepsy, and dementia.

Primarily, the pain caused by multiple fat tumors can be continuous or paroxysmal in nature, aggravates on movement, and can last from few minutes to several hours. Patient commonly presents with depression, generalized weakness, and irritability. The disorder is chronic and can gradually progress resulting in serious medical complications like myxedema (severe hypothyroidism-decreased thyroid hormone level), early congestive heart failure, hypertension (high blood pressure), extensive joint pain, tremors, cyanosis (bluish discoloration of the skin), headaches, paroxysmal flushing episodes, and epistaxis (bleeding through nose).

The major cardinal symptoms of this disease include multiple lipomas and associated neuropathic pain. Other symptoms of Dercum's disease include fatty deposits, obesity, easy formation of bruises, impaired memory, weakness, sleep disturbances, depression, fatigue, anxiety, impaired concentration, shortness of breath, rapid heartbeat, diabetes, constipation, bloating, including muscle and joint aches.

What are the types of Dercum's disease?

Based on the widely spreading pain, various types of Dercum's disease can be diagnosed which are as follows:

Type I: Also known as the juxta-articular type of dercum's disease which is characterized with painful, fat folds over the hips and within the knees. Rarely, it is accompanied by fat growths in the upper-arm.

Type II: Also called a diffuse, generalized type that is characterized with widespread pain due to a fatty growth. Unlike type I variety, it causes painful fat growths in various fat bearing areas of the body such as the dorsal upper-arm fat, the stomach wall, soles of the feet, dorsal fat folds, and in the axillary and gluteal regions.

Type III: Also called as the nodular type of lipomatosis which is associated with intense pain around different parts that bear multiple lipomas. It may also occur in individuals without generalized obesity. Clinically, these lipomas measure between 0.5-4 cm, have soft consistency, and are attached to the adjacent tissue.

References: http://www.genome.gov/17516629

http://en.wikipedia.org/wiki/Adiposis_dolorosa

http://www.webmd.com/a-to-z-guides/dercum-disease

http://www.emergingworlds.com/ch_conditions_detail.cfm?vPageid=157

http://emedicine.medscape.com/article/1082083-treatment#showall

http://emedicine.medscape.com/article/1082083-clinical

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Author: Dr Chaitra S

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