Adiposis Dolorosa (Dercum Disease) Clinical Presentation

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

History

Previously healthy women notice lumps or previously present lumps start growing. They describe pain and discomfort in the region of the lumps, associated with weakness. Before the onset of the disease, the patient is usually only slightly overweight, but, in a short time, obesity ensues. The pain increases with the subsequent increase in fatty tissue and in connection to menstruation. Pain is also worsened with palpation. [1]

The painful lipomas have been reported to occur in any location. [29] It was previously thought that the head and the neck were excluded, but it is now suspected that retrobulbar fat deposits may cause facial pain in some patients.

The first classification system for the disease was developed in 1900 by Giudiceandrea and was subsequently amended by Roux and Viteat, among others. [11, 30] Most recently, a 2012 review proposed the following classification system [13] :

  • Type I: Generalized diffuse form; generalized, widespread painful adipose in the absence of discreet lipomas

  • Type II: Generalized nodular form; widespread painful adipose with concomitant intense pain in and around multiple discreet lipomas

  • Type III: Localized nodular form; pain in and around multiple discreet lipomas

  • Type IV: Juxta-articular form; discreet deposits of excess fat in specific locations, including at the medial aspect of the knee, the hips, and, rarely, the upper arm

The pain varies from discomfort on palpation to excruciating, paroxysmal spontaneous attacks. [9] The pain can be aching, burning, or stabbing, often described by the patient as "it hurts everywhere." The pain is usually symmetrical; however, it can become localized to the thighs, the knees, or the upper extremities. Pain can be felt in the skeletal system and in the fat.

Hyperalgesia is found by light pressure and touch in the fatty tissue below the skin and is made worse by tightly fitting clothes or showering. The pain is temperature and weather dependent; it decreases in dry heat and when pressure is high. Hot baths can have a positive but short-term effect in the relief of pain, but some patients do not tolerate heat.

Other symptoms, with variable incidence

Problems with mobility may occur for large masses located on the lower extremities. [31]

The fingers have a tendency to swell up, fumble, and tingle, and they can be numb (paresthesias), in addition to secondary median nerve compression.

General tiredness similar to the symptoms of chronic fatigue syndrome may be present. Light physical activity and poor sleep aggravate the tiredness.

A tendency to bruise, possibly secondary to the formation of delicate vessels in fat deposits, may be present. Coagulation test results are normal.

Morning stiffness and stiffness after resting may occur (arthralgia).

Headaches (eg, tension headaches, classic migraine, neck headaches) may occur. Also, pain in the jaw and the eyes due to retrobulbar fatty tissue may be present.

Cognitive dysfunction, with concentration and memory problems, may be present.

Dizziness, paresthesia, and motor impairment were described in a case of a 52-year-old woman, with the authors suggesting that neurological and neuropsychiatric manifestations are likely a relevant and probably underestimated component to the disease. [32]

Bouts of depression (atypical depression, possibly latent) may occur; this finding is not associated with the onset of the disease. This association, among others, has been challenged given the prevalence of depression among obese individuals. However, a 2012 study of 111 patients with Dercum disease (adiposis dolorosa) compared with obese control subjects revealed a statistically significant greater prevalence of depression among patients with disease. [10]

Feeling hot affects a small number of patients, with recurring high temperatures of 37.5-39°C for weeks at a time associated with worsening of pain.

Patients may become susceptible to infection, as with one patient who developed sepsis secondary to steatonecrosis. [33] Pain is exacerbated with infections.

Others may include constipation, bloating, muscle aches, and sleep disturbances. [34]

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Physical Examination

Clinical examination is key for diagnosis. A characteristic physical examination finding is pain that is out of proportion. Palpation of the nodules reveals exquisitely tender subcutaneous nodules, and the most common areas involved include proximal extremities, trunk, and buttocks. [1] Passive and active movement of joints should not increase or decrease the amount of pain. [2]

Dercum disease (adiposis dolorosa) patients are obese (usually 50% over the normal weight for their age). In some patients, only localized fat, without general obesity, is present.

Lipomas are multiple, painful, symmetrically distributed, fatty deposits that are either diffuse or localized. The abdominal region and the lower extremities are common sites, especially around the knees. The ankle is an uncommon site of involvement. [35]

Hyperalgesia is found in the fatty tissue below the skin on light pressure and touch.

Other findings include acral swelling, bruises, and telangiectasias.

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Complications

Dercum disease (adiposis dolorosa) can be debilitating and can lead to incapacitation. Also, because Dercum disease symptoms are nonspecific, unnecessary medical procedures, tests, and operations can result in several complications.

Although rare, septicemia leading to septic shock, following necrosis of a fatty tumor, has been reported. [36]

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