How is Dercum disease managed?

Management of Dercum's Disease (Adiposis Dolorosa)

Dercum's disease should be managed with a multidisciplinary approach focused on pain control as the primary outcome, with treatment options including analgesics, liposuction for painful deposits, and adjunctive therapies targeting associated symptoms.

Clinical Presentation and Diagnosis

• Dercum's disease is characterized by painful subcutaneous adipose tissue deposits, typically occurring in overweight or obese individuals, most commonly between ages 35-50 years, and is 5-30 times more common in women than men 1
• Minimal diagnostic criteria include generalized overweight/obesity combined with painful adipose tissue 1
• Associated symptoms include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, fatigue, and joint aches 1, 2
• Diagnosis requires systematic physical examination and thorough exclusion of differential diagnoses including fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, and adipose tissue tumors 1

Classification

Dercum's disease can be classified into four subtypes:

• Type I: Generalized diffuse form - diffusely widespread painful adipose tissue without clear lipomas 1, 3
• Type II: Generalized nodular form - general pain in adipose tissue with intense pain in and around multiple lipomas 1, 3
• Type III: Localized nodular form - pain in and around multiple lipomas 1, 3
• Type IV: Juxtaarticular form - solitary deposits of excess fat near joints, such as the medial aspect of the knee 1, 3

Treatment Approach

Pain Management

• Analgesics should be the first-line treatment for pain control, though no specific analgesic regimen has demonstrated complete and long-lasting pain relief 1, 2
• Lidocaine (local or systemic) has shown some efficacy in reducing pain in patients with Dercum's disease 1, 2

Surgical Interventions

• Liposuction has demonstrated pain reduction in selected patients and should be considered for those with well-defined painful lipomas not responding to conservative management 1, 4
• Surgical excision of particularly painful lipomas may provide symptomatic relief, though recurrence is common 4, 5

Pharmacological Options with Limited Evidence

• Medications that have shown some benefit in case reports include:
  • Methotrexate and infliximab 1
  • Interferon α-2b 1
  • Corticosteroids 1
  • Calcium-channel modulators 1

Management of Associated Symptoms

• Depression and anxiety, which commonly accompany Dercum's disease, should be addressed with appropriate psychiatric evaluation and treatment 1, 2
• Sleep disturbances may require specific management strategies as they significantly impact quality of life 1, 3

Practical Considerations

• The pain in Dercum's disease tends to remain relatively constant over time, necessitating long-term management strategies 1
• Due to the rarity of this condition, patients may be misdiagnosed as pain-seeking or having psychological disorders, leading to inadequate treatment 4
• Treatment should involve a multidisciplinary team specializing in chronic pain management 1
• No single treatment has demonstrated revolutionary results or complete pain resolution, so management should focus on improving quality of life and functional status 1, 2

Monitoring and Follow-up

• Regular follow-up is essential to assess treatment efficacy and adjust management strategies accordingly 2
• Patients should be monitored for development of new painful deposits that may require additional intervention 5
• Psychological support should be ongoing as many patients experience significant impact on mental health and quality of life 1, 4