Adiposis dolorosa

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at August 3, 2016
StartDiseasesAdiposis dolorosa

Adiposis dolorosa is an autoimmune disease that mostly affects women between the ages of 25 and 60. Also known as Dercum disease, this disorder similarly affects males in all age groups. It is a recurring condition characterized by mild to severe pain of the fatty tissue. Other scientific names for this condition include juxta-articular adiposis dolorosa, lipomatosis dolorosa, morbus Dercum’s, and fatty tissue rheumatism.

Contents

Definition & Facts

In 1882, adiposis dolorosa was first documented in a medical journal by an American neurologist known as Francis Xavier Dercum. Adiposis dolorosa is a rare illness characterized by growth and swelling of adipose which is fat tissue. The growths or lipomas occur just below the skin (subcutaneously) and may appear on the upper legs, upper arms, and on the posterior.

Pain caused by adiposis dolorosa can sometimes be chronic. The pain is triggered when nerves squeeze the growths. In rare cases, lethargy, weight gain, depression, and confusion may be experienced by the patient.

The exact cause of adiposis dolorosa is unknown. However, it is often associated with obesity, especially in postmenopausal women aged between 45 and 60. Nevertheless, the condition has been reported in minors. The prevalence of the disease in the world is unknown since it is under-diagnosed. There are four types of adiposis dolorosa including:

  • Type I -This type is characterized by pervasive occurrence of aching lipomas in a diffused manner.
  • Type II – In this type of adiposis dolorosa, there is presence of nodular lipomas with critical pain.
  • Type III – Like type II, the type II is also a nodular type but its occurrence is localized to a particular part of the body.
  • Type IV – In type IV, the occurrence of lipomas appear near joints especially around the hips and knees.

Symptoms & Complaints

Many adiposis dolorosa patients experience soreness on various parts of their bodies. The soreness may last for hours and may come and go or recur. In severe cases, movement aggravates the pain. Fatty tissues are found on most parts of the body except in the head and neck.

A few individuals with the illness experience inflammation on various parts of the body, particularly the hands. The swelling frequently occurs without any cause and vanishes without being treated. Substantial weight gain is a common symptom of adiposis dolorosa.

Other symptoms include stiffness after resting especially in the morning, general weakness, tendency to bruise easily, fatigue, irritability, and headache. Difficulty concentrating, susceptibility to infections, and bouts of depression have been reported in rare cases.

Adiposis dolorosa has also been associated with congestive heart failure, arthritis, high blood pressure (hypertension), dry eyes, sleep disorders, lip inflammation and nose inflammation, mental deterioration and myxedema, a condition caused by an underactive thyroid (hypothyroidism).

Causes

Many medical professionals believe that adiposis dolorosa occurs when the body’s immune system attacks healthy tissue. Problems with metabolism and problems with the functioning of the endocrine system have also been associated with the condition.

Some medical professionals believe that the condition may be genetic. A small number of cases stem from genetic mutations that are hereditary. However, most cases involve patients without a family history of the disorder.

Diagnosis & Tests

Diagnosis of adiposis dolorosa relies on physical examination. The disease’s basic diagnostic criteria include generalized obesity and severe pains in the adipose tissue that may last up to three months.

Many related disorders including lipedema, fibromyalgia, panniculitis, and endocrine diseases such as hypothyroidism and Cushing's syndrome have the same symptoms as Dercum’s disease. Like adiposis dolorosa, they are also associated with obesity and pain, and they must be ruled out when rendering a diagnosis.

Patients with excessive accumulation of fat in the upper torso, head and neck region may be diagnosed with Lanois-Bensaude syndrome (also known as benign symmetric lipomatosis and Madelung’s disease)

Treatment & Therapy

Adiposis dolorosa is primarily treated through pain management though approaches relying on pain medications have many side effects. Some analgesics (painkillers) can be used to manage pain including lidocaine and nonsteroidal anti-inflammatory drugs (over-the-counter medicines like ibuprofen and naproxen). Opiate painkillers may also be used to treat pain but should be prescribed and taken with caution due to the risk of opiate dependence.

Corticosteroids such as prednisone have also been used to treat patients with the condition. They may be administered intravenously. However, corticosteroids should be used with care because some medical practitioners believe they can worsen and, in some cases, even cause the condition.

Surgical removal of fatty tissues around the joints can temporarily reduce the pain, but reappearance may occur. Some adiposis dolorosa patients have used liposuction as a supplementary treatment. Liposuction provides primary reduction of pain and enhances the patient’s quality of life. Another form of treatment for adiposis dolorosa is integrative therapies like massage, hydrotherapy, and acupuncture.

Prevention & Prophylaxis

It is often difficult to tell how this disorder can be prevented since it has no known cause. Nevertheless, studies indicate that making certain lifestyle changes such as having a healthy diet and regular exercise can help prevent the condition.

The rare adipose disorder (RAD) diet is another approach that can be used to prevent adiposis dolorosa. The diet focuses on easing inflammation and controlling the condition. The RAD diet includes consuming foods rich in healthy carbohydrates with low glycemic index, protein, and whole foods. It also involves avoiding processed foods, animal fats, carbohydrates, and refined sugars.