What are the treatment options for Dercum's disease?

Treatment of Dercum's Disease

For Dercum's disease (adiposis dolorosa), treatment should focus on pain management through a combination of topical lidocaine patches, pregabalin for neuropathic pain, manual lymphatic drainage, and surgical excision of symptomatic lipomas, as no single therapy provides complete long-lasting pain relief. 1

Diagnostic Confirmation First

Before initiating treatment, confirm the diagnosis by:

• Systematic physical examination identifying multiple painful subcutaneous lipomas, typically on trunk and extremities 1
• Exclusion of fibromyalgia, lipoedema, panniculitis, endocrine disorders, and primary psychiatric disorders 1
• Ultrasound or MRI to visualize lipomatous masses 2

Classification-Based Treatment Approach

Dercum's disease should be classified into four forms to guide management 1:

Type I - Generalized Diffuse Form:

• Diffusely widespread painful adipose tissue without clear lipomas 1
• Prioritize systemic pain management over surgical intervention

Type II - Generalized Nodular Form:

• General adipose tissue pain with intense pain in/around multiple lipomas 1
• Combination of systemic therapy and selective surgical excision

Type III - Localized Nodular Form:

• Pain confined to multiple lipomas 1
• Surgical excision is most appropriate

Type IV - Juxtaarticular Form:

• Solitary deposits near joints (e.g., medial knee) 1
• Surgical removal to relieve mechanical symptoms 2

First-Line Pain Management

Topical Lidocaine 5% Patches (Lidoderm):

• Apply directly over painful lipomas 3
• Demonstrated >60% pain reduction (from 8/10 to 3/10) sustained over multiple months 3
• Safe, non-invasive option preferable to systemic medications 3

Pregabalin:

• Effective for neuropathic pain component from nerve compression by enlarging lipomas 4
• Combine with manual lymphatic drainage for optimal results 4
• Traditional analgesics and NSAIDs typically have minor or no effect 4

Adjunctive Physical Therapy

Manual Lymphatic Drainage:

• Successful when combined with pregabalin 4
• Addresses tissue edema and mechanical pressure 4

Surgical Intervention

Liposuction or Surgical Excision:

• Provides pain reduction but not complete or permanent relief 1
• Useful for ameliorating local symptoms, particularly in Type III and IV disease 5
• Recurrence is common, as seen in a case with recurrence 2 years post-excision 2
• Should be performed for symptomatic lipomas causing mechanical arthralgia or significant pain 2

Additional Pharmacologic Options

The following have shown some benefit but lack robust evidence 1:

• Methotrexate and infliximab - for inflammatory component
• Interferon α-2b - mechanism unclear
• Corticosteroids - short-term use only
• Calcium-channel modulators - limited data

Multidisciplinary Pain Management

Because no treatment provides revolutionary or long-lasting complete pain reduction, patients should be managed by chronic pain specialists 1:

• Pain medicine physicians for medication optimization
• Physical therapy for lymphatic drainage and mobility
• Psychiatric support for associated depression and anxiety (common comorbidities) 1, 2
• Surgical consultation for symptomatic lipoma removal

Important Caveats

• Pain in Dercum's disease remains relatively constant over time without spontaneous resolution 1
• The condition predominantly affects women (5-30 times more common than men), typically presenting between ages 35-50 1
• Associated symptoms include easy bruising, sleep disturbances, memory impairment, depression, fatigue, and joint pain 1
• Treatment is palliative rather than curative; set realistic expectations with patients 1