Dercums Disease (Adiposis Dolorosa) (Morbus Dercum)
First described in the literature by Francis Xavier Dercum in 1892, Dercum’s disease (aka Dercum disease, Adiposis Dolorosa or Morbus Dercum) is described as 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. It is also described in the following way: a rare disorder in which there are fatty deposits which apply pressure to the nerves, resulting in weakness and pain. Various areas of the body may swell for no apparent reason. The swelling may disappear without treatment, leaving hardened tissue or pendulous skin folds. It is a chronic disease with mild to intensive pain in the fatty tissue, fatigue and obesity as the most apparent symptoms, althouch some patients are not obese. The disease affects the entire body and can present a long list of symptoms. This disorder usually occurs in postmenopausal 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. Dercums is characterized by multiple painful lipomas (benign fatty tumors) that arise in adult life. The fatty tumors are most often located on the trunk and limbs with sparing of the face and hands.
The cause of Dercums is unknown. It seems to have an autosomal dominant pattern of inheritance no associated gene has been identified thus far. According to the latest research, Dercum is an auto-immune disease such as rheumatism, and not a metabolic disturbance, as was believed earlier. I doubt this also.
- Pain in the fatty tumors lasting for at least three months
- Often obesity developing over a short period of time *but not always)
- 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.
- Some affected individuals may experience depression, lethargy, and/or confusion.
- Lipomas can be felt in the fat, they are intensely painful to pressure, and usually harmless, unless a tumor moves to the lung or heart which can be fatal. 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.
- Pain is chronic and increases with the years, but varies 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 the face and hands. Severe 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.
- Many have difficulties going to sleep because of the pain, but other forms of sleeping disorders also occur.
- Easy bruisability is common.
- Stiffness often occurs after resting, especially in the mornings.
- Feeling hot is frequent in some, and there may be a mild fever several weeks in a row, with increased pain and incapacity to work as a consequence. The cause of this fever is 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.
- Skeletal pain can occur 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 intellectually demanding jobs.
- 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 Dercums implies a long, chronic pain syndrome of debilitating nature. The pain can make it difficult to carry out normal activities. On physical exam pain palpation of and pressure on the subcutaneous lipomas should raise suspiciion of the diagnosis.
- Oral mexiletine, may also be effective in eliminating pain for variable periods of time.
- Surgical excision of fatty tissue deposits around joints (liposuction) has been used in some cases. It may temporarily relieve symptoms although recurrences often develop.
- The use of infliximab and methotrexate has been proposed.
- 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.
- Medical cannabis seems to be very effective in ameliorating pain.