About juxta-articular adiposis dolorosa

What is juxta-articular adiposis dolorosa?

Dercum's disease is an extremely rare disorder characterized by multiple, painful growths consisting of fatty tissue (lipomas). These growths mainly occur on the trunk, the upper arms and upper legs and are found just below the skin (subcutaneously). Pain associated with Dercum's disease can often be severe. Pain may be caused by these growths pressing on nearby nerves. Dercum's disease mainly occurs in adults and more women are affected than men. In some cases, affected individuals may also experience weight gain, depression, lethargy, and/or confusion. The exact cause of Dercum's disease is unknown.

What are the symptoms for juxta-articular adiposis dolorosa?

The characteristic finding of Dercum’s disease is the formation of multiple, painful growths consisting of fatty tissue (lipomas) that are found just below the surface of the skin but also deep. Lipomas in people with Dercum’s disease may be found in any part of the body, although they are rare in the head, neck, hands and feet. The trunk, upper arms and upper legs are most often affected. The lipomas can be the size of a pea or as large as a grape, plum or fist. Dercum’s disease may occur in one or more people in a family with familial multiple lipomatosis (FML Type), may occur due to angiolipomas which are known to be painful (angiolipoma type) or may occur as small diffuse (widespread) pea-sized or larger lipomas anywhere in subcutaneous fat tissue especially over the ribs and including around joints due to the presence of Inflammation in the body from any cause (healing disorder type). Pain may vary from mild discomfort when a growth is pressed or touched to severe Pain that is disproportionate to the physical findings; Pain must be present for at least three months. Some affected individuals feel that “all fat hurts.” Pain can last for hours and may come and go or last continuously. In severe cases, Pain may worsen with movement. Many people with Dercum’s disease describe exacerbations or “flares” of Pain that may be accompanied by worsening of symptoms such as Fatigue and “brain fog”. The exact reason for Pain associated with Dercum’s disease is unknown but may occur because the lipomas press on nearby nerves or because the fascia is inflamed. The lipomas associated with Dercum’s disease have more connective tissue than non Dercum’s disease fat. Connective tissue (fascia) in fat has more Pain sensors than muscle, for example, and when tight or inflamed, can cause Pain as in fibromyalgia. Fibromyalgia is not uncommon in people with Dercum’s disease.

Some individuals with Dercum’s may experience Swelling of various areas of the body, especially the hands, but often one arm or leg may be swollen and therefore more painful. Swelling occurs for no apparent reason, though on imaging, the lymphatic system can be slow to pump in the painful limb compared to the less painful limb, or frank lymphedema may be present. The Pain may disappear without treatment but any manual therapy such as massage or manual lymphatic drainage can help. Significant weight gain is a common occurrence for most individuals affected by Dercum’s disease and needs to be treated to avoid progression to pre-diabetes or diabetes.

Additional symptoms may occur in individuals with Dercum’s disease including fatigue, generalized weakness, a tendency to bruise easily, headaches, irritability, and Stiffness after resting, especially in the morning, gastrointestinal symptoms, palpitations and shortness of breath. An association with bouts of depression, memory or concentration problems, anxiety, and a susceptibility to infection has been noted in some cases.

Additional reports in the medical literature have linked Dercum’s disease to a variety of conditions including arthritis, high blood pressure (hypertension), congestive heart failure, sleep disorders, dry eyes, and myxedema, a condition due to an underactive thyroid that is characterized by dry skin, Swelling around the lips and nose, mental deterioration and infection.

What are the causes for juxta-articular adiposis dolorosa?

The exact cause of Dercum’s disease is unknown. In most cases, Dercum’s disease appears to occur spontaneously for no apparent reason (sporadic), though causes of inflammation should be sought. One case reported the development of Dercum’s disease in a man after trauma (motor vehicle accident).

Some reports in the medical literature have suggested that Dercum’s disease may be an autoimmune disorder- a disorder in which the body’s immune system mistakenly attacks healthy tissue. Disturbances in endocrine function and improper breakdown (metabolism) of fat have also been proposed as potentially playing a role the development of the disorder. One case reported in the medical literature was linked to the use of high-doses of corticosteroids and some cases have been linked to infection including Lyme disease or Valley fever (coccidioidomycosis).

Some cases of Dercum’s disease have run in families and several reports in the medical literature cite the possibility that the disorder may be inherited as an autosomal dominant trait in these cases (FML type or angiolipomatosis type). Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother.

Dominant genetic disorders occur when only a single copy of an altered gene is necessary for the appearance of the disease. The altered gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the altered gene from affected parent to offspring is 50 percent for each pregnancy. The risk is the same for males and females.

What are the treatments for juxta-articular adiposis dolorosa?

No specific treatment exists for Dercum’s disease. Treatment is directed toward the specific symptoms that are apparent in each individual and is primarily focus on easing the characteristic painful episodes.

Various painkillers (analgesics) have been tried with limited effectiveness. Injections of corticosteroids have also been used to treat individuals with Dercum’s disease. However, in one reported case in the medical literature, the use of high-doses of corticosteroids was linked as a possible cause of the disease. Intravenous administration of the pain reliever lidocaine and/or ketamine may provide temporary relief from pain in some people.

Surgical excision of lipomas may temporarily relieve symptoms although the generation of inflammation (part of the healing process) during and after the surgery may induce more lipomas to develop in that area. Liposuction has been used as a supportive treatment for some individuals with Dercum’s disease and may provide an initial reduction in pain and improvement in quality of life. These effects may lessen over time.

Psychotherapy and consultation with pain management specialists may be helpful for enabling affected individuals to cope with long-term intense pain. Other treatment is symptomatic and supportive.

What are the risk factors for juxta-articular adiposis dolorosa?

Dercum’s disease affects females more often than males with medical publications citing the disease as 5-30 times more common in women. Dercum’s disease can affect individuals of any age with an average age of 34 years in one medical publication. Although an extremely rare occurrence, it has been reported in children. The prevalence of Dercum’s disease is unknown. The disorder is underdiagnosed, making it difficult to determine its true frequency in the general population. Dercum’s disease was first described in the medical literature in 1882 by an American neurologist named Francis Xavier Dercum.

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