This book describes Dercum’s Disease (Adiposis Dolorosa), Diagnosis and Treatment and Related Diseases
Dercum’s disease is a rare disorder that produces painful growths of fatty tissue called lipomas in subcutaneous fat tissue.
It is also known as Adiposis dolorosa, Ander syndrome, adipose tissue rheumatism, adiposalgia, or lipomatosis dolorosa.
This disorder normally involves the torso, upper arms, or upper legs.
Different areas of the body may swell for no apparent cause.
The swelling may vanish without treatment, leaving hardened tissue or pendulous skin tags.
Some doctors have lobbied for a “minimal definition” of Dercum’s disease (adiposis dolorosa), which was recently proposed to include these:
1. Generalized obesity
2. Chronic pain (>3 mo) in the adipose tissue
Criteria for diagnosis
There are 4 cardinal symptoms of Dercum’s disease (adiposis dolorosa), and these remain the standard for diagnosis of classic disease:
1. Multiple, painful, fatty masses
2. Generalized obesity, normally in menopausal age
3. Asthenia
4. Neuropsychiatric disorders, such as emotional instability, depression, epilepsy, confusion, and dementia
Dercum’s disease is anywhere from 5 to 30 times more frequent in women.
Cause
The underlying cause of Dercum’s disease remains unknown.
Possible causes have been indicated, but none have been confirmed.
These have involved long-term treatment with:
1. High-dose corticosteroids;
2. Endocrine system abnormalities;
3. Nervous system dysfunction;
4. Mechanical pressure on nerves, adipose tissue dysfunction;
5. Trauma; and
6. Changes in fatty acid or carbohydrate metabolism.
Researchers have also indicated that it could be an autoimmune disorder which is a disease that produces the immune system to mistakenly attack healthy tissue.
Others believe it is a metabolic disorder related to not being able to properly break down fat.
Since the disorder has rarely happened in more than one person within a family, it may have a genetic component.
No specific gene known to be linked with the disorder has been identified.
The origin of the pain linked with the disorder is also poorly understood.
It is believed that fatty deposits produce nerve compression, resulting in weakness and pain.
Most cases of Dercum’s disease are sporadic (not inherited).
This means that it normally happens in people with no family history of the disorder.
In some of these cases, it seems to have been inherited in an autosomal dominant route.
In these instances, when an affected person has children, each child has a 50% (1 in 2) risk to inherit the gene causing the disorder.
Dercum’s disease is classified into four stages:
I. Generalized diffuse form – a type of disease with diffusely present painful lipomas
II. Generalized nodular form – a type of disease with the evidence of general pain in and in the neighboring tissue of multiple lipomas.
III. Localized nodular form – a type of disease with the present pain in and around the lipomas
IV. Juxtaarticular form – a type of disease with solitary lipomas at the internal parts of the knee.
The most prominent symptoms are:
1. Sleep disorder,
2. Anxiety,
3. Depression,
4. Cognitive impairment,
5. Tachycardia,
6. Respiratory disorders and
7. Gastrointestinal malfunction.
Dercum’s disease is the same as fibromyalgia but with the presence of painful lipomas.
The presence of pain is needed for an adequate diagnosis.
The treatment normally focuses on pain treatment:
Pain relievers
Cortisone
Methotrexate
Interferon alpha
Other treatments are:
Surgical removal of lipomas
Liposuction
Intravenous lidocaine
Cognitive behavioral therapy
TABLE OF CONTENT
Introduction
Chapter 1 Dercum’s Disease
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Fibromyalgia
Chapter 8 Lipoma
Epilogue
