Dercum's Disease: Symptoms, Diagnosis, and Treatments

• May 21, 2024
• By Fernando Amato

Dercum's Disease is a rare condition characterized by obesity and painful adipose tissue. Symptoms, diagnosis, and treatments.

Dercum's Disease, also known as adiposis dolorosa, is a rare condition characterized by generalized obesity or overweight in combination with chronic painful adipose tissue. It is a differential diagnosis for lipedema. Therefore, we will explore the main aspects of this disease, including its classification, symptoms, diagnosis, treatment, and prognosis.

• 📌 Clear definition: Generalized obesity and painful adipose tissue.
• 📌 Detailed classification: Four distinct forms of presentation.
• 📌 Varied symptoms: Include pain, bruising, sleep disturbances, and psychiatric problems.
• 📌 Multiple treatments: Ranging from liposuction to calcium channel modulators.
• 📌 Constant prognosis: Persistent pain over time.

Classification of Dercum's Disease

Dercum's Disease is classified into four distinct forms:

1. Generalized Diffuse Form: Characterized by widely disseminated painful adipose tissue without the presence of clear lipomas.
2. Generalized Nodular Form: Presents generalized pain in adipose tissue and intense pain around multiple lipomas.
3. Localized Nodular Form: Characterized by pain around multiple lipomas.
4. Juxta-Articular Form: Includes excessive fat deposits in specific areas, such as the medial aspect of the knee.

Symptoms Associated with Dercum's Disease

In addition to pain in adipose tissue, other common symptoms of Dercum's Disease include:

• Fat deposits: Accumulation of adipose tissue in different parts of the body.
• Easy bruising: Tendency to develop bruises easily.
• Sleep disturbances: Problems falling asleep or staying asleep.
• Impaired memory and difficulty concentrating: Cognitive problems that affect the quality of life.
• Depression and anxiety: Psychiatric symptoms that frequently accompany the condition.
• Tachycardia and shortness of breath: Associated cardiovascular symptoms.
• Diabetes, swelling, constipation, fatigue, and weakness: Other symptoms that may be present.

Diagnostic Criteria

Experienced chronic pain specialists diagnose Dercum's Disease clinically.

Diagnostic criteria include:

1. Generalized obesity or overweight.
2. Chronic pain in adipose tissue for more than three months.

Therefore, the diagnosis must be made after excluding other conditions, such as fibromyalgia, lipedema, panniculitis, endocrine disorders, and primary psychiatric disorders.

Almost 40% of Dercum's patients, however, present with some autoimmune pathology, such as rheumatoid arthritis and systemic lupus erythematosus.

Treatment and Management

There is no definitive treatment for Dercum's Disease, but several approaches can help reduce pain:

• Liposuction: Can provide pain relief by removing painful fat deposits.
• Analgesics and lidocaine: Include options such as intravenous lidocaine and lidocaine patches for pain relief.
• Methotrexate and infliximab: Medications that have shown some benefit in isolated cases.
• Interferon α-2b and corticosteroids: Other treatments that may help some patients.
• Calcium channel modulators: Medications like pregabalin used to treat neuropathic pain.
• Rapid-cycle hypobaric pressure: Technique that can reduce pain in some patients.

Prognosis

Pain in Dercum's Disease tends to be constant over time, although intensity may vary. Patients, therefore, generally need continuous and multidisciplinary management to control symptoms.

Frequently Asked Questions

What is Dercum's Disease?

Dercum's Disease, or adiposis dolorosa, is a rare condition characterized by generalized obesity and painful adipose tissue.

What are the main symptoms of Dercum's Disease?

The main symptoms, therefore, include pain in adipose tissue, fat deposits, easy bruising, sleep disturbances, memory problems, depression, and anxiety.

How is Dercum's Disease diagnosed?

Diagnosis is clinical, therefore, based on generalized obesity or overweight and chronic pain in adipose tissue for more than three months, after excluding other conditions.

What treatment options are available?

There is no cure, but treatments such as liposuction, analgesics, lidocaine, methotrexate, infliximab, interferon α-2b, corticosteroids, and calcium channel modulators can help reduce pain.

Is Dercum's Disease more common in any specific group?

Yes, the disease, therefore, is more common between 35 and 50 years old and affects women five to thirty times more than men.

What are the possible causes of Dercum's Disease?

Proposed causes include nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction, and trauma, but none have been confirmed.

What is the prognosis for patients with Dercum's Disease?

Pain, therefore, tends to be constant over time, and continuous and multidisciplinary management is necessary to control symptoms.

Are there genetic factors involved in Dercum's Disease?

Most cases occur sporadically, but there are reports of autosomal dominant inheritance with variable expression.

Can Dercum's Disease be confused with other conditions?

Yes, conditions such as fibromyalgia, lipedema, panniculitis, and endocrine disorders, therefore, may have similar symptoms and should be excluded before diagnosis.

Which specialists are indicated to treat Dercum's Disease?

Specialists in chronic pain, internal medicine, rheumatology, and plastic surgery are indicated for treatment.

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Reference: Hansson E, Svensson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012;7:23. doi: 10.1186/1750-1172-7-23.

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