May 14, 2019
Dercums Disease – A Case Report
PhD MSc BSc
THE COLLEGE OF
A case presentation describing a rare disease with multi-system involvement, resulting in non-lifestyle caused obesity and painful lipomas around the body.
A patient knowledgeable of his own disease presented at Holme Valley Memorial Hospital requesting surgery to his painful heel.
This case report discusses the assessment, imaging and surgical pathway undertaken for his care and reviews the current literature for the causes, diagnosis and management of Dercum’s Disease
Dercum’s disease is a rare, complex disorder involving multiple painful growths in subcutaneous adipose tissue.
It was eponymously named after Francis Dercum, an American neurologist, in 1888, and 130 years later it remains poorly understood. The World Health Organization has recognised it as a distinct disease entity and classes it as lipomatosis not elsewhere classified. It is also known as adiposis dolorosa, and the name
portends its clinical characteristics, the English translation being ‘painful fatty deposits.’
The aetiology of Dercum’s disease is unknown. Various causes have been suggested, including nervous system, endocrine, lymphatic and vascular dysfunction, adipose tissue dysfunction, mechanical pressure on nerves and traumainduced.
Altered lipid metabolism is championed as a cause within the literature, with authors demonstrating altered formation in long fatty-acid chains and disrupted conversion of glucose to triglycerides.5 Genetic causes have been discussed in the literature, with mixed views.
A definite hereditary element has been discussed,1 and some authors have suggested it is inherited on an autosomal dominant basis with variable penetrance, whereas others have indicated it is due to a new mutation with no relevant family history. Some studies have indicated that Dercum’s lipomas are indistinguishable from common lipomas Hansson et al compared fat samples from healthy obese control patients with fat samples from Dercum’s patients.
An inflammatory response was found in the adipose tissue but no more so than in the non-Dercum’s control group, but the study was flawed by a lack
of information on oral anti-inflammatory drug use, which may have affected the results.
Other histological findings include fat cells being larger and the ratio of monounsaturated fat and unsaturated fat being greater in Dercum’s disease. Heat created by the fat cells is greater than that of normal obese tissue.
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