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neuropeptide Y is on the lower side of normal, and B-endorphin is on the higher side (H. Brorson, B. Fagher, R. Ekman; unpublished data).

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.

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.

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]

Treatment & Management

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Media Gallery

Multiple painful lipomas. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/w/dercum-1.jpg).

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Author

Laura F McGevna, MD Assistant Professor of Medicine, Dermatology Division, University of Vermont College of Medicine

Laura F McGevna, MD is a member of the following medical societies: American Academy of Dermatology, Dermatology Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Hannah J Porter, MD, MBA, MS Resident Physician, Department of Dermatology, University of Vermont Medical Center

Hannah J Porter, MD, MBA, MS is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Catharine Lisa Kauffman, MD, FACP Georgetown Dermatology and Georgetown Dermpath

Catharine Lisa Kauffman, MD, FACP is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Royal Society of Medicine, Society for Investigative Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Acknowledgements

Tammie Ferringer, MD Dermatopathology Section Head, Dermatopathology Fellowship Director, Departments of Dermatology and Pathology, Geisinger Medical Center

Tammie Ferringer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose. Nada Macaron, MD Consultant Pathologist, Institute of Pathology and Laboratory Medicine, Sheikh Khalifa Medical city, UAE

Nada Macaron, MD is a member of the following medical societies: College of American Pathologists and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Marjan Yousefi, MD Department of Dermatology, Geisinger Medical Center

Marjan Yousefi, MD is a member of the following medical societies: American Academy of Dermatology and Phi Beta Kappa

Disclosure: Nothing to disclose.