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Adiposis Dolorosa - Causes, Symptoms, Risks, Diagnosis, and Treatment

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An uncommon condition known as adiposis dolorosa results in painful lipomas, which are fatty tissue growths. It is also known as Dercum’s disease.

Medically reviewed by

Dr. Kaushal Bhavsar

Published At June 20, 2023
Reviewed AtDecember 1, 2023

What Is Adiposis Dolorosa?

Adiposis dolorosa is characterized by painful folds of fatty tissue (adipose tissue) or the development of many lipomas, which are benign, noncancerous fatty tumors. Most women with this disorder are obese or overweight, and signs and symptoms usually start to show between the ages of 35 and 50.

Abnormal fatty tissue, or lipomas, can develop anywhere on the body in those with adiposis dolorosa, but they are most frequently detected on the torso, buttocks, upper arms, and legs. Usually, lipomas feel like hard nodules under the skin. If the growths are pressing on a neighboring nerve, they might produce intense burning or pain. Some people have intermittent pain, while others experience ongoing suffering. Pain may get worse with movement or pressure on adipose tissue or lipomas. Lipomas occasionally make it difficult to move normally.

What Are the Causes of Adiposis Dolorosa?

Some of the current hypotheses are adipose tissue malfunction, lymphovascular pathology, mechanical pressure on nerves, nervous system dysfunction, and trauma-induced. A few case reports of people with adiposis dolorosa have been thought to be autosomal dominant with varying phenotypic manifestations. The majority of occurrences of the illness are found to be sporadic and lack any particular genetic abnormalities.

What Are the Symptoms of Adiposis Dolorosa?

Adiposis dolorosa symptoms can differ from person to person. However, nearly all adiposis dolorosa patients have slow-growing, painful lipomas. A lipoma can be as big as a human fist or as little as a pebble. While some people's lipomas vary in size, others have only one size, the lipomas. Adiposis dolorosa-related lipomas frequently hurt when touched, probably because they are pressing on a nerve. Some people experience constant pain.

Other symptoms of adiposis dolorosa include:

  • Weight gain.

  • Depression.

  • Weakness.

  • Bruising.

  • Headache.

  • Irritability.

  • Rapid heart rate.

  • Fatigue.

  • Swelling.

  • Unable to concentrate.

  • Memory problems.

  • Shortness of breath.

  • Constipation.

How Is Adiposis Dolorosa Diagnosed?

The age at which symptoms may first develop varies depending on the condition. Some diseases' signs and symptoms might appear at any age. Knowing when symptoms first appeared can aid doctors in making the right diagnosis. Adiposis dolorosa cannot be diagnosed using any set criteria.

Instead, the doctor will probably concentrate on excluding alternative diagnoses like fibromyalgia or lipedema. The doctor might do a lipoma biopsy to accomplish this. To do this, a little tissue sample must be examined under a microscope. Additionally, they might employ an MRI or CT scan to aid in their diagnosis. The doctor may classify it based on the location and size of the lipoma.

  • Mixed - It is the combination of both small and large lipomas.

  • Diffuse - Small lipomas are widespread.

  • Nodular - Large lipomas are usually around the back, arms, thighs, and abdomen.

What Are the Histopathological Findings of Adiposis Dolorosa?

According to histology, biopsies collected from patients with adiposis dolorosa exhibit an inflammatory response with lymphocytes and macrophages. It is possible that the inflammation in this condition is not any worse than it would be in a healthy patient with obesity who does not have adiposis dolorosa.

What Is the Differential Diagnosis of Adiposis Dolorosa?

In cases of generalized, diffuse forms of adiposis dolorosa, other conditions with general pain, such as lipoedema, fibromyalgia, panniculitis, endocrine disorders involving pain and obesity, and psychiatric conditions affecting pain, particularly in conjunction with obesity, should be considered in the differential diagnosis.

Other multiple lipoma syndromes, such as multiple symmetric lipomatosis, familial multiple lipomatosis, myoclonic epilepsy with red ragged fibers (MERRF) syndrome, multiple endocrine neoplasia type 1 (MEN1), and neurofibromatosis type 1, should be considered in the differential diagnosis of nodular forms of adiposis dolorosa. Sarcomas and other adipose tissue tumors must be ruled out in cases with isolated lesions.

Adiposis dolorosa cannot be diagnosed using any set criteria. Instead, the doctor will probably concentrate on excluding alternative diagnoses like fibromyalgia or lipedema. The doctor might do a lipoma biopsy to accomplish this. A little tissue sample must be examined under a microscope in a biopsy. Additionally, they might employ an MRI or CT scan to aid in their diagnosis.

How Is Adiposis Dolorosa Treated?

There is no established standard of care for this disease, even though several therapeutic alternatives have been tried. To relieve symptoms and foster support, therapies are tailored to specific symptoms in each patient. Weight loss has proven to have no impact on lipomas; in a few instances, it may even worsen symptoms. Pain management may be necessary if the pain is incapacitating and intermittent. According to case studies, analgesics, liposuction, lipectomy, and manual lymphatic drainage are all viable pain management strategies.

Traditional analgesics cannot fully control the pain in adiposis dolorosa, while non-steroidal anti-inflammatory medications (NSAIDs) and narcotics are shown to reduce pain. Ketamine, Lidocaine, Methotrexate, corticosteroids, Infliximab, transcutaneous electrical stimulation, interferon alpha-2b, calcium-channel modulators, and rapid cycling hypobaric pressure are some other drugs and therapy modalities with conflicting evidence of possible effectiveness. Short-term pain relief may be achieved with lipectomy and liposuction, but the discomfort usually returns, and the quality of life is not significantly improved.

Who Is At Risk for Adiposis Dolorosa?

Men can have adiposis dolorosa, but women are 20 times more likely to be diagnosed if they are obese, middle-aged, or have undergone menopause. It typically manifests between the ages of 45 and 60.

How Is the Prognosis of Adiposis Dolorosa?

Adiposis dolorosa frequently persists for a long period and can potentially worsen with time. Nevertheless, there have been case reports with inconsistent prognostic outcomes.

Conclusion:

Adiposis dolorosa is characterized by the development of many painful lipomas (benign, fatty tumors). Lipomas can develop anywhere on the body and can be extremely painful. Weakness and memory issues could also be symptoms. Women are more frequently impacted than males are, and it typically affects adults. The precise reason is not known. Although rare familial cases with autosomal dominant inheritance have been reported, most cases are sporadic (not inherited).

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Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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