Adiposis Dolorosa (Dercum Disease) Workup

Updated: Aug 25, 2021
  • Author: Laura F McGevna, MD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

Results of hormonal studies to rule out Cushing syndrome, thyroid abnormalities, and other endocrinologic abnormalities are characteristically normal.

Dercum disease (adiposis dolorosa) patients might have associated slight-to-moderate elevation of cholesterol levels.

Erythrocyte sedimentation rate results may be slightly elevated.

Coagulation test results usually are normal.

In spite of obesity, hypertension and type 2 diabetes mellitus are often absent.

An increase in certain active parameters is seen in the following sedimentation rate; alpha-1-antitrypsin; orosomucoid (alpha-1-acid glycoprotein, an acute phase reactant); haptoglobin; and complement factors C3, C4, Clq, and Cls. [14, 15]

The heat produced by the fat cells when measured with a microcalorie meter is approximately twice as high as that taken from people who are extremely overweight.

The ratio of monounsaturated fatty acid (16:1, 18:1) in the fatty tissue is greater than that of saturated fatty acid (14:1, 18:0) shown by a comparison with healthy people in controls. [37, 39]

The levels of substance P in the cerebrospinal fluid is significantly lower compared with healthy weight-matched controls. However, the average in both cases is above the normal level. [40] The level of the neuropeptide Y is on the lower side of normal, and B-endorphin is on the higher side (H. Brorson, B. Fagher, R. Ekman; unpublished data).

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Imaging Studies

Ultrasonography and MRI may aid in the diagnosis of Dercum disease (adiposis dolorosa). [35, 41, 42]

On MRI, the lesions appear oblong, and this may be due to septal distortion that is seen on histopathological evaluation. In a study by Tins et al of 13 patients with Dercum disease, lesions of the condition were found to be markedly hyperechoic on ultrasonography, superficial in location, and distinct from characteristic lipomas. [43] Further, when validated on more than 6,000 MRIs, they appeared as ill-defined, nodular, “blush-like” subcutaneous fat on unenhanced MRI with a decreased T1-weighted signal. No case of Dercum disease was without these features in the study, and the authors concluded that these findings, along with multiple subcutaneous fatty lesions, is “very suggestive and possibly pathognomonic” for the condition.

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Histologic Findings

A review of histopathologic findings did not reveal any significant features that might distinguish Dercum disease (adiposis dolorosa) tumors from common sporadic lipomas. An inflammatory response with lymphocytes and macrophages is seen on biopsy, although the inflammation may not be any more pronounced than that of a healthy patient with obesity without a diagnosis of Dercum disease. [1] Minor features that were detected include a slight accumulation of perivascular lymphocytes and plasma cells and extremely large fat cells compared with those of healthy controls of similar weight. The tumors can be encapsulated, or the fatty deposits can be diffuse.

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Other Tests

Bioimpedence has been described as a noninvasive tool that may help to distinguish Dercum disease (adiposis dolorosa) from lipedema. Regional bioimpedence measures (tissue water bioimpedence in the upper and lower extremities) seem to differ between Dercum disease and lipedema. [44]

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