What Is Dercum’s Disease?

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What Is Dercum’s Disease?

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Dercum’s disease is recently either referred to as Adiposis dolorosa, Anders’ syndrome, lipomatosis dolorosa, or Dercum-Vitaut syndrome. Dercum’s disease is a rare connective tissue condition that is characterized by the growth of lipomas.

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 Lipomas are non-malignant tumors comprising of fat cells; it is a tumor found abnormally growing in fatty tissues and contains fatty cells. In a case of Dercum’s disease, painful growths of lipomas (abnormal fatty tissues) are found mostly in the torso (trunk), upper arms, or upper leg (thigh region).

These abnormal fatty tissues mostly can grow subcutaneously (just under the skin) and also can be found growing deeper in the body, forming within muscles, tendons, ligaments, or bone.

Dercum’s disease is categorized as a connective tissue disease because fatty tissues are classified as connective tissues. Severe pain is closely associated with Dercum’s disease; pain may be as a result of lipomas pressing against nearby nerves or inflamed connective tissue, medically referred to as fascia, commonly associated with lipomas.

Dercum’s disease is a chronic condition, that is, it is a long-lasting condition that progresses with time. The etiology and mechanism of Dercum’s disease remain a mystery but history dictates that the disease first appeared and was described at Jefferson Medical College by a young neurologist Francis Xavier Dercum in 1892, from which the disease inherited its name.

Global activity and prevalence of Dercum’s disease is not very known but based on individual cases and according to a review in the Orphanet Journal of Rare Disease (OJRD), Dercum’s disease is an obesity condition more frequent in women than men, although the condition occurring in men is not totally ruled out.

It occurs mostly in ages ranging between 35 to 60 years. The condition is mostly diagnosed in women who are obese, middle-aged, or have gone through menopause. Children rarely get Dercum’s disease. 

Dercum’s disease is subdivided into four types based on the distribution of the affected adipose tissue (fatty tissues of the body) in association with lipomas.

The types of Dercum’s disease include:

  • Generalized diffuse: characterized by a widespread painful adipose tissue with a small size or no lipomas at all.
  • Localized nodular: mostly common type, this type is associated with painful adipose tissue exclusively with and around fairly large-sized lipomas, around the arms, back, abdomen or thighs.
  • Generalized nodular: generalized painful adipose tissue, more intense within and around lipomas, a combination of both large and lipomas around the body.
  • Juxtaarticular: painful solitary fat deposition in the proximity of large joints.

Causes of Dercum’s Disease

The source and mechanism of Dercum’s disease are still not known. The origin of the pain associated with the condition is esoteric and the disease clinically is likely to be identified by its symptoms than by its physiological or metabolic characteristics. The fatty deposits (lipomas) results in nerve

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compression that causes generalized or localized pain and body weakness. Possible hypothesis as regards the cause of Dercum’s disease include:

  • Central Nervous System dysfunction,
  • Adipose tissue dysfunction,
  • Mechanical disruption of nerves,
  • Trauma,
  • Use of corticosteroids,
  • Infection including Lyme disease or valley fever.
  • Endocrine system dysfunction or disturbance
  •  Genetic origin

A genetic cause is considered because Dercum’s disease seems to run in some families, and in some occurrences, the disease seems to be inherited as an autosomal dominant trait (one out of a person’s two copies of the gene, inherited from a parent, is faulty). However, most reported cases of this condition are sporadic; the only case to occur in the family.

Also, some research actually suggested that the disease is an autoimmune disorder, that is, a condition that occurs when the healthy body tissues are attacked by the body’s immune system. However, the single cause of Dercum’s disease has not been accurately identified.

Symptoms of Dercum’s Disease

Symptoms vary from person to person. However, most commonly occurring symptom in persons with Dercum’s disease is painful, fatty lipomas that grows slowly in size. 

Lipomas may appear all over the body especially the trunk as in some cases or may be localized to some parts of the body, such as the head, neck, hands, and feet.

The trunk, upper leg, and upper arm are mostly affected and the size of the lipomas may range from the size of a pea to as large as a grape or fist.

Dercum’s disease lipomas appear in numbers, in some patients, the lipomas may exist the same size while in others, the sizes may vary.

The lipomas are painful when pressed as the pain may be constant for some patients or paroxysmal, lasting for hours and worsens with movements.

Other symptoms associated with Dercum’s disease may include:

  • Obesity (weight gain)
  • Fatigue
  • Weakness, either localized or general
  • Swellings appear and disappear in different parts of the body, often on the hands.
  • Depression and emotional instability associated with epilepsy and confusion.
  • Easily bruised.
  • Problems with concentration, thinking, and memory.
  • Headaches
  • Insomnia
  • Body stiffness, especially in the morning after waking up.
  • Joint arches
  • Increased and rapid heart rate
  • Apnea
  • Constipation and gastrointestinal disorders

Medical literature has published additional reports have been linked Dercum’s disease to a variety of conditions including arthritis, congestive heart failure, dry eyes, epistaxis, and myxedema (a condition due to an underactive thyroid, characterized by dry skin, swelling around lips and nose, infection and medical deterioration). 

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Diagnoses of Dercum’s Disease

Dercum’s disease diagnoses are done through a physical examination of the patient. In order for physicians to confirm the diagnosis, all other possible differential diagnoses must first be eliminated.

The basic known criteria for Dercum’s disease are presentations of chronic pain in adipose tissue (body fat) and patients who are also obese.

There are no specific lab tests for Dercum’s disease but to exclude all other possible differential diagnoses, the doctor might conduct a biopsy on one of the lipomas; this entails taking a tissue sample and observing the sample under a microscope. Although ultrasound and MRI (magnetic resonance imaging) can play a role in diagnosis.

Differential Diagnosis

A disease or a medical condition must be confirmed through series of diagnosis and differential diagnoses of other secondary conditions that may present similar symptoms as the disease of primary focus must be excluded. Comparisons may come in handy during a differential diagnosis.

Some other conditions that may present very similar symptoms like Dercum’s disease may include:

Madelung’s disease

This condition is also known as multiple symmetric lipomatosis, it is a rare disease that affects uncoupling protein-1 and possibly brown fat tissue.

The condition has a similar symptom of fat deposits (lipomas) around the neck, shoulders, upper arms, and upper back. Male alcoholics are mostly affected, although women and non-drinkers can also develop Madelung’s disease.

Fibromyalgia

This is a chronic disorder of pain throughout the muscles of the body and presents abnormal episodes of fatigue. Other symptoms similar to Dercum’s disease are stiffness and muscular spasms with pain; that progresses gradually.

In addition, small specific areas are known as “tender points” usually found at the back of the neck, shoulders, knees, elbows, and hips are typically painful when pressure is applied to them.

Other symptoms include diarrhea, constipation, lack of sleep, chest pain, difficulty in concentrating, and painful urination.

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Familial Multiple Lipomatosis (FML)

This is also a rare but genetic disorder characterized by the formation of multiple lipomas at most often the trunk arms and thigh. The size and number of lipomas also vary like in the case of Dercum’s disease.

Individuals suffering from this condition may develop as much as a hundred small lipomas that are asymptomatic (causing no symptoms associated with pain) and are often found in similar locations on one side of the body compared to the other side (symmetric). Unlike Dercum’s disease, FML does not usually affect the neck and shoulders.

Angiolipomatosis

Angiolipomatosis is characterized by multiple lipomas with an increased number of blood vessels that contain fibrin clots and larger numbers of mast cells.

Just like Dercum’s disease, angiolipomatosis also present lipomas associated with chronic pain and this result in confusion between both conditions, hence people with angiolipomas are said to have Dercum’s disease-angiolipomatosis type.

This condition may also be inherited in an autosomal dominant manner.

Other disease that may also be similar in symptoms (development of benign tumors containg fatty cells) to Dercum’s disease may include:

  • Gardner syndrome,
  •  Proteus syndrome,
  • Lipedema,
  • Panniculitis,
  • Adipose tissue tumors,
  • Primary psychiatric disorder and
  • PTEN hamartoma syndrome.  

Treatment on Dercum’s Disease

The primary focus of Dercum’s disease treatment approaches is to easy symptoms as there is no universal cure for the disease.

In most cases, patients benefit from a combination of these treatment, and these involve complete cooperation with a doctor and a pain management specialist to find the safest and most suitable treatment-combo that would be effective for a patient.

Treatments include:

  • Surgical removal of lipomas
  • Liposuction (suction attempts of extra fat)
  • Pain relievers administration
  • Cortisone injection, methotrexate, interferon alpha, infilximab, intravenous lidocaine, calcium channels modulators; diuretics prescribed to reduce swelling and get rid of extra body water.
  • Electrotherapy
  • Acupuncture
  • Non-steroidal anti-inflammatory drugs
  • Staying healthy with an improved lifestyle could help with outlook.

Summary

Doctors can inform patients about some factors that tend to aggravate symptoms, which may include stress, not getting enough sleep, a poor diet, and engaging in very strenuous physical activities or exercises.

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Dercum’s disease might be difficult to diagnose and treat and by the time the condition is confirmed, it may be too late. Severe pain caused by Dercum’s disease can also lead to depression and addiction.

A pain management specialist should always be employed in a case of Dercum’s disease. You might also find an online or in-person support group for persons with rare diseases.

Sources;

Dercum’s Disease

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What Is Dercum’s Disease?

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