Ali Abbasian, MD, FRCPC

A 74-year-old female presented with multiple, soft swellings around the knees and upper thighs. These had developed slowly over five years and were extremely tender to pressure. Standing and walking was increasingly painful. Marked fibromyalgia was also identified

Answer: Juxta-articular adiposis dolorosa (Dercum’s Disease)

On physical examination, the patient was found to have extremely tender fatty areas around both knees and over both hip joints. She was moderately overweight with a BMI of 27. She had a waddling gait and described severe pain on standing and walking.

First identified by Francis X. Dercum, in 1892, Dercum’s Disease (also Adiposis Dolorosa) is described as, "A disease accompanied by painful localized fatty swellings and by various nerve lesions. It is usually seen in women." The cause is unknown.

Symptoms include pain in the fatty areas lasting for at least three months. The pain in fat and skin can be intense and is described as aching, stabbing, smarting, or burning. Swellings consisting of irregularly shaped soft fatty tissue may occur in many areas of the body. These deposits occasionally disappear, leaving hardened lumpy or rope-like tissue or pendulous folds of skin. The pain is chronic and progressive. It can exist in the entire fatty tissue layer, but most commonly affected are the knees, trunk, forearms, and thighs, sparing the face and hands. Severe weakness has been emphasized as a feature by some. The pain increases even at gentle touch, and massage is unpleasant. Other symptoms:

•Memory lapses and concentration difficulties

•Difficulty wearing tight fitting clothes or taking a shower

The pain seems to depend on the temperature and the weather, and decreases in dry heat. The pain can make it difficult to: walk, drive a car, turn a water tap, lift items from shelves, carry bags, open heavy doors, or perform all types of house care.

The cause is unknown. Dercum’s Disease is thought to be inherited as an autosomal dominant genetic trait, particularly strong in the line grandmother-mother-daughter. 

The radiologist is often the first person to identify the disorder although, knowledge about the disorder is not common and many patients may be misidentified. Weight loss has little or no effect on the symptoms. The classic physical sign is extreme pain when one of the fatty areas is squeezed.

MRI is the preferred instrumental examination. Ultrasound can add to the diagnosis with fatty areas appearing like lipomas but not firm and very painful when pressed. The fatty areas can be seen even in the weight bearing X-ray.

1. Berenguer B, de la Cruz L, de la Plaza R. Liposuction in atypical cases. 

2. De Silva M, Earley MJ. Liposuction in the treatment of juxta-articular adiposis dolorosa. 

3. Hansson E. Liposuction in Dercum’s Disease. Clinical Studies Regarding Dercum’s Disease and the Effect of Liposuction. Monograph: Department of Clinical Sciences in MalmÃ¶ Lund University. Plastic and Reconstructive Surgery.  2011 SkÃ¥ne University Hospital, MalmÃ¶, Sweden.

4. Tins BJ, et al., Adiposis dolorosa (Dercum’s disease): MRI and ultrasound appearances. 

http://dx.doi.org/10.1016/j.crad.2013.05.004

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74-year-old female presented with multiple, soft swellings around the knees and upper thighs. 

Ali Abbasian, MD, FRCPC

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