Known by various names, the Dercum’s disease is a condition of rare occurrence in which numerous lipomas develop in the body which is severely painful and weakens the muscle. Dercum’s Disease or Adiposis Dolorosa is a chronic as well as progressive condition in which benign tumors of lipids called lipomas develop in the torso and the extremities that lie close to the torso of the body. Dercum’s Disease or Adiposis Dolorosa prominently affects females who are obese and past their menopause phase. Though, less common this condition can also affect non-obese individuals with prevalence over males.

Dercum’s Disease or Adiposis Dolorosa

Dercum’s Disease or Adiposis Dolorosa: An overview

Lipomas can be defined as non-cancerous capsules of fat that develop just below the skin and are thin and fibrous in structure. There occurrence is localized towards the thighs, neck, torso, armpits and upper arms but can also occur anywhere in the body. These can range from one to many in their count and are commonly found in obese individuals.

In general cases, these lipomas are usually small, soft and painless, but in the case of Dercum’s Disease or Adiposis Dolorosa these growths become painful which occur due to the pressing of the nearby nerves or inability of the skin to receive optimal blood supply. The pain that befalls is extreme and continuous with frequent spasms. Dercum’s Disease or Adiposis Dolorosa becomes worse with the advancement of age.

Types of Dercum’s Disease or Adiposis Dolorosa

The Dercum’s Disease or Adiposis Dolorosa is generally of four types:

  • Type I: This type of Dercum’s Disease or Adiposis Dolorosa is characterized by widespread occurrence of painful lipomas in a diffused manner.
  • Type II: This type of Dercum’s Disease or Adiposis Dolorosa marks the presence of nodular lipomas with severe pain.
  • Type III: It is also a nodular type but the occurrence is localized to a specific part of the body.
  • Type IV: It is marked by the occurrence of lipomas near the joints especially in knees and hip regions.

Symptoms of Dercum’s Disease or Adiposis Dolorosa

The symptoms that help in the identification of Dercum’s Disease or Adiposis Dolorosa are categorized as cardinal symptoms and other potential symptoms. However, there is much ambiguity regarding the prominence of either type of symptoms.

  • Cardinal Symptoms of Dercum’s Disease or Adiposis Dolorosa: These includes symptoms like -
    • Painful and fatty lipomas.
    • Severe fatigue and weakness.
    • Obesity
    • Presence of depression, mental and emotional instability, perplexity, dementia and epilepsy.
  • Other Potential Signs and Symptoms of Dercum’s Disease or Adiposis Dolorosa: The other probable symptoms of Dercum’s Disease include -
    • High heart rate
    • Pain in the joints
    • Difficulty in sleeping
    • Easy bruising
    • Loss of focus and concentration along with memory issues.
    • Difficulty in catching of breath.

Epidemiology of Dercum’s Disease or Adiposis Dolorosa

Dercum’s Disease or Adiposis Dolorosa is known to appear in over eighty five percent of post-menopausal females. The age group affected by the disease usually ranges between thirty five to fifty years of age. Dercum’s Disease or Adiposis Dolorosa is also known to affect males but research shows that males are thirty to fifty times less prone towards the disease.

Prognosis of Dercum’s Disease or Adiposis Dolorosa

The prognosis of Dercum’s Disease or Adiposis Dolorosa marks the persistence of the condition throughout the lifetime. The pain can be lessened by taking pain relievers and cortisone shots. However, these are a source of temporary comfort. In long term view, prescription drugs along with holistic treatments can be taken to ensure better quality of life for patients suffering from Dercum’s Disease or Adiposis Dolorosa.

Causes of Dercum’s Disease or Adiposis Dolorosa

The underlying causes for Dercum’s Disease or Adiposis Dolorosa are still unknown but there are certain speculation regarding the reasons behind it. These are:

  • Possibility of an autoimmune disorder.
  • Disruption in the endocrine functioning of the body.
  • Administration of high doses of corticosteroids.
  • Heredity and inheritance of defective genes.

Nevertheless, it has been confirmed that Dercum’s Disease or Adiposis Dolorosa does not occur because of genetic mutation.

Pathophysiology of Dercum’s Disease or Adiposis Dolorosa

The pathogenesis and mechanism behind the Dercum’s Disease or Adiposis Dolorosa still remains unidentified. The cause behind the pain is unclear but it is assumed to occur as a result of fatty acid deposits that strain the underlying nerves. The theories also suggest the familial inheritance of multiple lipomas, autoimmune condition or alteration of fat metabolism due to excess of corticosterones in the body.

Risk Factors of Dercum’s Disease or Adiposis Dolorosa

The factors that pose a risk for Dercum’s Disease or Adiposis Dolorosa are:

  • Presence of obesity post menopause.
  • Age between late thirties to late fifties.
  • Presence of prior disorders like Cowden syndrome, Gardener’s syndrome, etc.
  • Familial history of lipomas.

Complications of Dercum’s Disease or Adiposis Dolorosa

The common complications that come up as a result of Dercum’s Disease or Adiposis Dolorosa are:

  • Swelling in the skin along with looseness and loss of elasticity.
  • Numbness and burning sensation in hands and feet.
  • Impaired function of heart or lungs due to the interference of lipomas in their optimal functioning.

Diagnosis of Dercum’s Disease or Adiposis Dolorosa

Diagnosis of Dercum’s Disease or Adiposis Dolorosa is tough as there are no defined tests to diagnose the same. The only approach for the identification and diagnosis of Dercum’s Disease or Adiposis Dolorosa is the observation of the symptoms over a period of at least three months. The lipomas can also be felt by the physician when palpated.

Management of Dercum’s Disease or Adiposis Dolorosa

There is no defined and documented cure for Dercum’s Disease or Adiposis Dolorosa. The treatment mainly focuses on the reduction of the pain, symptoms and complications that arise due to the condition. The treatments include:

  • Medications to Manage Dercum’s Disease or Adiposis Dolorosa: The common pain relieving medications that are prescribed include NSAIDs, Lidocaine injections and acetaminophens.
  • Surgical Procedures to Manage Dercum’s Disease or Adiposis Dolorosa: The surgical procedures aim at removal of lipomas and fat from the body thereby efficiently reducing pain and improving the quality of life. One such procedure used is liposuction.
  • Integrative Therapies to Treat Dercum’s Disease or Adiposis Dolorosa: Though these therapies do not have much clinical evidence with respect to treatment of the condition but these immensely help in providing temporary relief to the existing symptoms. Such therapies are hydrotherapyacupuncture and massages.

Lifestyle and Coping for Dercum’s Disease or Adiposis Dolorosa

The general approach that can be followed as coping mechanism for Dercum’s Disease or Adiposis Dolorosa is following the RAD (Rare Adipose Disorder) diet. This diet aims at reducing the inflammation and effective management of the condition, though there are not many evidences to support the same. RAD diet involves following a diet rich in whole foods, healthy proteins and carbohydrates with low glycemic index. It also involves simultaneous limiting of food that constitutes Advanced Glycation End products (AGEs) like packed foods, refined sugars and carbohydrates, animal fats which are known to cause inflammation.

There are also other remedies that can help in reduction of pain associated with Dercum’s Disease or Adiposis Dolorosa like bathing in Epson salts, mustard seeds and sodium bicarbonate.

Prevention of Dercum’s Disease or Adiposis Dolorosa

There are currently no proofs and studies available, that support the prevention of the Dercum’s Disease or Adiposis Dolorosa.

Conclusion

Dercum’s Disease or Adiposis Dolorosa is an uncommon condition which involves abrupt occurrence of lipomas in the torso, arms and hip regions. These are very painful which tends be constant even with passage of time. The cause behind the disease is still unknown but the studies support the prevalence of the condition among obese post-menopausal women. Due to lack of research regarding Dercum’s disease, the diagnostic measures and treatments appropriate for Dercum’s Disease or Adiposis Dolorosa are still unknown. The treatment for Dercum’s Disease or Adiposis Dolorosa generally involves observation of symptoms over time and providing treatments to reduce them which generally involved prescribing pain medication and following healthy diet to limit lipid accumulation. Some cases may also require lipid removal via surgery, to reduce the pain and inflammation.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: July 1, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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