Adiposis dolorosa

Dercum's disease is the formation of multiple fatty deposits in various sites.

This is a rare disease that predominantly affects women over men in a ratio of up to 30:1 (1)

• most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men.
• prevalence of Dercum's disease has not yet been exactly established.

This condition may be familial but the majority of cases are sporadic.

It is characterized by the formation of circumscribed painful adipose tissue deposits in the subcutaneous tissues of the extremities and other parts of the body. The most common site for the deposits is the knees. The size of lesions varies from 0.5 to 5 cm. Patients are often obese.

The disease may be associated with emotional instability, fatigue, weakness and, very occasionally, dementia.

Histological examination reveals no abnormalities of the adipose tissue. There is no fat necrosis as seen in panniculitis. Giant cell formations may be seen.

There is no satisfactory treatment

• the following treatments have lead to some pain reduction in patients with Dercum's disease:
  • liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon a-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure
  • none of the treatments have led to long lasting complete pain reduction (1)

Reference:

• Hansson E et al. Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012 Apr 30;7:23.