What Causes Dercum’s Disease?

Dercum’s Disease Explained: Causes, Painful Lipomas & Care Strategies

Adiposis dolorosa (Dercum’s disease) is a rare disorder characterised by numerous, painful fatty tumours (lipomas) which usually develop in middle-aged adults. Though it is unclear what causes this condition, current research proposes several potential mechanisms and risk factors.

This article discusses the definition, causes, symptoms, and more details about Dercum’s disease. Keep reading for a detailed insight.

What is Dercum's disease?

Dercum’s disease is a chronic adipose tissue disorder manifested by:

• Painful fat nodules (lipomas) under the skin of the trunk, arms, and legs
• Often accompanied by obesity, chronic fatigue, and cognitive difficulties
• Mainly develops in adults, particularly in women aged 35 to 50

Although it has severe symptoms, it usually does not affect life expectancy.

What are the Causes of Dercum’s Disease?

The exact cause of Dercum’s disease remains unknown, but proposed mechanisms include:

• Genetic Predisposition: In most cases, it develops sporadically, although some families have autosomal dominant inheritance.
• Inflammation of Adipose Tissue and Nerve Compression: Lipomas can lead to chronic pain with compression of local nerves or fascial inflammation.
• Endocrine or Metabolic Dysfunction: It is associated with abnormal metabolism of fats and potential hormonal imbalances.
• Immune-Mediated Mechanisms: There is some theoretical evidence of an autoimmune element, but the evidence is inconclusive.
• Triggering Factors: Corticosteroid use, trauma, or infections (including Lyme disease) are reported to be associated with disease onset.
• Obesity: Obesity is also widely linked with the condition, although it is not a direct cause.

What are the symptoms of Dercum’s disease?

The symptoms can vary from person to person, but common signs include:

• Painful Lipomas: These fatty lumps usually develop on the torso, upper arms, and upper legs and cause continuous or occasional pain.
• Chronic Fatigue: Many individuals experience extreme fatigue and weakness.
• Emotional Disturbances: Depression and anxiety are also prevalent.
• Swelling and Tenderness: Injured areas can be tender to the touch and may easily bruise.
• Stiffness: Particularly upon waking or after a nap.
• Headaches and Irritability: Some individuals report frequent headaches and mood changes.

How is Dercum's Disease Diagnosed?

Diagnosis is clinical, based on history and physical exam to rule out other conditions like lipedema or fibromyalgia. Additional methods include:

• Clinical Evaluation: Doctors assess the presence of painful lipomas, fatigue, cognitive issues, and other symptoms.
• Imaging Studies: Ultrasound or MRI (magnetic resonance imaging) scans may be used to examine lipomas and ensure they are benign.
• Biopsy: In some cases, a tissue biopsy may be performed to confirm the nature of the fatty growths.
• Exclusion of Other Conditions: Since Dercum’s disease shares symptoms with disorders like lipedema and fibromyalgia, tests may be conducted to rule out these possibilities.

What are the treatment approaches for Dercum’s disease?

Though incurable, treatment focuses on symptom relief and improved quality of life, including:

1.  Pharmacological Options

• Pain Relief: Acetaminophen, NSAIDs (non-steroidal anti-inflammatory drugs), opioids, pregabalin, mexiletine, low-dose steroids, interferon α, and infliximab.
• IV Lidocaine Infusions: May offer temporary relief lasting hours to months.
• Additional Agents: Methotrexate, d-amphetamine, and ketamine can be used experimentally.

2.  Procedural and Surgical Options

• Liposuction or Excision: Reduces lipoma size and pain; often relapses but may last years.
• Lidocaine Injections: Provide local pain relief for several months.
• Electrotherapy: Techniques like transcutaneous electrical stimulation, hydromassage may reduce pain.
• Manual Lymphatic Drainage: Helpful, but may accelerate lipoma growth in some individuals.

What are the care strategies for Dercum's disease?

Dercum's disease is a chronic condition with no known cure, but managing symptoms and improving quality of life is possible through various care strategies, such as:

• Multidisciplinary Approach: Check-up with endocrinologists, dermatologists, pain specialists, and mental health professionals.
• Pain Management: Tailored therapy plans combining medications, procedures, and lifestyle strategies.
• Physical Therapy & Gentle Exercise: Supports function and mobility.
• Psychological Support: Cognitive behavioural therapy (CBT) and counselling help address chronic pain and emotional burden.
• Prevention Strategies: Limited to avoiding known triggers; no established prevention protocols due to unknown aetiology.

What are the complications of Dercum’s disease?

Dercum's disease can lead to several chronic and debilitating complications, affecting both physical and mental well-being. Here are some of the key risks:

• Pain and Disability: Without treatment, persistent pain may impair daily functioning.
• Emotional and Mental Health Impact: Higher risk of depression and cognitive issues.
• Medication Side Effects: Potential for adverse reactions to steroids, opioids, or immunomodulators.

What is the Role of Health Insurance in Dercum’s Disease?

Treatment involves multi-pronged interventions, imaging, specialist consultations, medications, and surgical procedures.

Although the exact cause of Dercum’s disease remains unknown, research suggests a multifactorial origin, including genetic, metabolic, inflammatory, and possibly autoimmune or endocrine influences. This rare condition leads to multiple painful lipomas and systemic symptoms, requiring comprehensive management.

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