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Adiposis dolorosa
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Adiposis dolorosa

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Contributors: Chris G. Adigun MD, Jeffrey D. Bernhard MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Adiposis dolorosa, also known as Dercum disease, is an uncommon condition characterized by the development of numerous painful subcutaneous lipomas. The condition predominates in postmenopausal, often obese, women. The painful lipomas are symmetrically distributed and may be either diffuse or localized. The trunk and lower extremities, especially around the knees, are the most commonly affected sites; however, with the exception of the head and neck, the fatty deposits have been reported to occur in any location.

In addition to being painful, it is also common for paresthesia of the overlying skin of the individual lipomas to be present. Degree of pain experienced increases with body mass index (BMI), and most patients are 50% above the normal weight for their age. Other associated findings include hyperalgesia to light pressure, acral swelling, telangiectasia, and bruising. Complaints of weakness and fatigability, as well as psychiatric disturbances such as depression, confusion, and dementia, are common. When the lipomas arise around joints, affected individuals may experience mechanical arthralgia.

The pathogenesis of the condition is unknown, although metabolic studies suggest that alterations in fatty acid metabolism and peripheral insulin resistance play a role, while other studies suggest that it is a primary autoimmune process.

Patients suffering from adiposis dolorosa often experience severe, chronic pain. This places them at risk for numerous unnecessary diagnostic procedures and even surgery. In addition, there is often an absence of laboratory or other radiographic abnormalities, as well as lack of response to treatment, which leads to these patients being labeled as having an anxiety disorder or other psychological problems. It is therefore important to keep this condition in mind when evaluating a patient with chronic pain.

For more information, see OMIM.

Codes

ICD10CM:
E88.2 – Lipomatosis, not elsewhere classified

SNOMEDCT:
71404003 – Lipomatosis dolorosa

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Last Updated: 09/06/2017
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Adiposis dolorosa
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Adiposis dolorosa : Painful skin lesions, Large fatty tumors
Clinical image of Adiposis dolorosa
A large subcutaneous tumor on the upper arm.
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