Adiposis dolorosa
Adiposis dolorosa, also known as Ander's disease or Dercum's disease, is a rare disease characterized by multiple painful lipomas that arise in adult life. Older medical literature states that "the disease occurs most often in obese postmenopausal women, but can also occur in men." Surveys of current sufferers seem to indicate that the disease causes the obesity. It also occurs in men and women of all ages. The fatty tumors are most often located on the trunk and limbs with sparing of the face and hands. It was first formally described by Dr. Francis Xavier Dercum in 1888.
Dercums Disease/Adiposis Dolorosa
First identified by Dr. Dercum, in 1892, Dercum's Disease (also Dercum Disease or Adiposis Dolorosa) is described several ways in the literature. Here are two:
"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." (Dorland, 27th ed) [This definition is found in National Library of Medicine: IGM Metathesaurus Information.]
Paraphrasing the National Organization of Rare Diseases (NORD):
"Dercum's 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 the body may swell for no apparent reason. The swelling may disappear without treatment, leaving hardened tissue or pendulous skin folds."
Morbus Dercum (Adiposis dolorosa) is a chronic disease with mild to intensive pain in the fatty tissue, fatigue and obesity as the most apparent symptoms. But the disease affects the entire body and can present a long list of symptoms. This disorder usually occurs in females between the ages of 25 and 60, but can début in all ages, as well as in younger people and amongst males. It is about 20 times more frequent in females than in males. Dercum's Disease was originally described in the medical literature in 1892 by the American doctor Frances Xavier Dercum.
Symptoms
Pain in the fatty tumors lasting for at least three months. Often obesity occurs in a short period of time. But there is also a type of Dercum's disease that can occur with normal weight. The pain in fat and skin can be very intense, and can be described as aching, stabbing, smarting or burning. Swellings consisting of irregularly shaped soft fatty tissue deposits may occur in many areas of the body. These deposits may spontaneously disappear, leaving hardened lumpy or rope-like tissue or pendulous folds of skin. The pain is chronic and increases with the years, but varies much in cycles. It can exist in practically the entire fatty tissue layer, but most commonly affected are the knees, trunk, forearms and thighs, sometimes sparing of the face and hands. Severe asthenia (weakness)has been emphasized as a feature by some. The pain is spontaneous and increases powerfully even at gentle touch, and massage can feel unpleasant. Some affected individuals may experience depression, lethargy, and/or confusion. Lipomas, "fatty tumors " can be felt in the fat, they are intensely painful, and usually harmless, unless a tumor moves to the lung or heart which can be fatal.
Other common symptoms are:
- Various areas of the body may swell for no apparent reason. The fingers becomes clumsy, a person may drop things and sometimes the fingers go numb.
- Disturbed sleep, many have difficulties going to sleep because of the pain, but other forms of sleeping disorders also occur.
- General fatigue, worsening with even mild activity.
- Tendency to become black and blue; this seems to arise spontaneously or after alleviated blow.
- Stiffness after resting especially in the mornings.
- Skeletal pain in wrist, elbows, hips, tail bone and the long bones of the arms and legs.
- Headache, usually a combination between tension headache and classic migraine.
- Memory lapses and concentration difficulties making it difficult to learn new things and to accomplish demanding intellectually jobs.
- Feeling hot is often felt by the patients, some have 37.5 to 39 Celsius degree fever several weeks in a row, with increased pain and incapacity to work as a consequence. The reasons are unknown.
- Tenderness under the feet, akin to walking on glass.
- Tenderness in the skin, difficulties in wearing tight fitting clothes or taking a shower.
- Infection sensitivity. Frequently increased pain during infections or active allergy attacks.
The pain seems to depend on the temperature and the weather and decreases normally at dry heat. Warm baths have a positive but temporary impact, though some patients do not tolerate heat. Generally increases pain in conjunction with menstruation. Sexual relation problems can arise because of the pain. Dercum's pain always exists, regardless if a person is asleep or awake. The diagnosis of Dercum's Disease implies a long, chronic pain syndrome of debilitating nature. The pain can make it difficult to: walk, drive a car, open the water tap, lift items from shelves, carry bags, open heavy doors, vacuum, hang laundry, wash floors, wipe windows etc.
Causes
The causes of the disease are poorly researched. The symptoms and the lack of treatments can seriously affect the quality of life. Since the disorder does not show on the outside it can be hard for others to see the level of difficulties and discomfort the patient experiences. More than half of the patients are unable to work. The disorder can grow slowly for many years or very quickly because of external stress, such as: surgery, pregnancy or flu. Dercums Disease is thought to be inherited as an autosomal dominant genetic trait, particularly strong in the line grandmother-mother-daughter. According to the latest research, Dercums Disease is an auto-immune disease such as rheumatism, and not a metabolic disturbance, that was believed earlier.
Diagnosis and treatment
The Dercums Disease diagnosis is based on what the patient tells and what the doctor finds at his examination. There are no tests to take, but some blood samples can show signs of infection and/or that the immune system are very active. The doctor can also take blood samples in order to exclude other diseases. The knowledge about the disorder is not well know and many patients are poorly treated. The treatment of Dercums Disease patients is inadequate. Attempts have been made to get patients to lose weight, but it is extremely difficult. If the patients succeed, the weight loss has little or no effect on the symptoms.
Treatment
Treatment methods include:
- Medication that decreases the pain and improves the sleep
- Massage
- Warmth and warm baths
- Acupuncture
- Practicing relaxation techniques
- Avoiding physical and psychological stress
Information about the disorder and how to live with it
Treatment of Dercums Disease is primarily directed at easing painful episodes. Surgical excision of fatty tissue deposits around joints may temporarily relieve symptoms although recurrences often develop. Intravenous infusions of the local anaesthetic drug lidocaine may give temporary relief from pain. Additional treatments of lidocaine may be necessary periodically to sustain the effect. Another analgesic drug, peroral mexiletine, may also be effective in eliminating pain for variable periods of time. Psychotherapy may be helpful for enabling affected individuals to cope with long-term intense pain. Other treatment is symptomatic and supportive.
Conclusions
Dercum's Disease causes obesity with painful fatty tumor and fatigue. It is a chronic, often very painful disease that has incapacitating consequences. This disease affects all aspects of a patient's life, patient education improves the outcome of the disease. Collaboration is needed and occasionally a big effort by the patient to learn to live with the disorder. Patients find that they can no longer do all that was easy before. People with Dercum's Disease feel pain for a number of reasons. Small actions, such as wringing a washcloth, opening a can or taking a shower, can be extremely painful experiences. It is important that Dercum's Disease patients receive reliable information about the disorder and support from the surrounding community in order to be able to live as positively as possible and that they find their own capabilities. This helps the patient to be less dependent on others, which is of major importance in both family and working life.
Resources:
http://dercums_data.tripod.com