Adiposis dolorosa
| Adiposis dolorosa | |
|---|---|
| Synonyms | Dercum's disease; Anders disease |
| Classification and external resources | |
| Specialty | endocrinology |
| ICD-10 | E88.2 |
| ICD-9-CM | 272.8 |
| OMIM | 103200 |
| DiseasesDB | 29660 |
| eMedicine | derm/839 |
| MeSH | D000274 |
| Orphanet | 36397 |
Adiposis dolorosa, also known as Dercum's disease or Anders disease, is a rare condition characterized by generalized obesity and fatty tumors in the adipose tissue. The tumors are normally painful and found in multiples on the extremities. The cause and mechanism of Dercum's disease remains unknown. Possible causes include nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction, and trauma.
Dercum's disease was first described at Jefferson Medical College by neurologist Francis Xavier Dercum in 1892.
Four cardinal symptoms have sometimes been used as diagnostic criteria:
There are also potential signs of the disease which are identified as the following:
However, as it is unclear which symptoms are cardinal and which symptoms are minor signs in Dercum’s disease, it is unclear which should be used as diagnostic criteria. Researchers have proposed a 'minimal definition' based on symptoms most often part of Dercum's disease: 1) Generalized overweight or obesity. 2) Chronic pain in the adipose tissue. The associated symptoms in Dercum’s disease include obesity, fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint and muscle aches. Regarding the associated symptoms in Dercum’s disease, only case reports have been published. No study involving medical examinations has been performed in a large group of patients.
There are no currently known causes of this disease. There are studies currently proposing several theories of the causes which include inflammation of the adipose tissue, nervous system malfunction and endocrine malfunction. None of the theories that are currently proposed have been found viable. Since little is known about Dercum's disease, there are currently no known modes of prevention. Some theories state that maintaining a healthy weight and diet can help prevent Dercum's although it has not been proven.
Dercum's disease can affect people of any gender and of any age. The majority of cases are linked to women between the ages of 45 and 60, that are overweight and postmenopausal. Due to the difficulty of diagnosis of this disease, many cases are underreported or misdiagnosed and it is difficult to understand what part of the population is affected by it the most.
There are currently no known mechanisms for this disease.
Diagnosis of Dercum's disease is done through a physical examination. In order to properly diagnose the patient, the doctor must first exclude all other possible differential diagnosis. The basic criteria for Dercum's disease is patients with chronic pain in the adipose tissue (body fat) and patients that are also obese. Although rare the diagnosis does not have to include obesity. Dercum's disease can also be inherited and a family medical history may aid in the diagnosis of this disease. There are no specific laboratory test for this disease. Ultrasound and Magnetic Resonance Imaging can play a role in diagnosis.
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