Dercum's Disease

What is Dercum's Disease?
Dercum's disease is a rare disorder characterized by multiple, painful growths consisting of fatty tissue (lipomas). Pain may be caused by the lipomas pressing on nearby nerves. Dercum's disease mainly occurs in adults and more women are affected than men.( 1)

Symptoms
• Painful lipomas in subcutaneous adipose tissues, present >3 months, upon palpation, sporadic or spontaneous.
• Cognitive: expressive language difficulties and memory deficits
• Vascular: angiolipomas, easy bruising, flushing
• Dermatologic: multiple cherry angiomas and petechiae
• Gastro-intestinal: GERO, IBS, early satiety
• Musculoskeletal: joint pain/stiffness increased in areas of fat deposits; muscle pain/stiffness pronounced at standing after period of inactivity and after physical activity
• Cardio/Pulmonary: tachycardia ranging from palpations to supraventricular tachycardia requiring pharmacologic intervention; shortness of breath
• General: sleep disturbance, insomnia, depression, anxiety, weight gain, heavy or prolonged menstrual bleeding, migraines, fatigue increased by ADLs and exercise
• Symptoms may progress to immobility

Note: Symptoms vary significantly by person; not all people have all symptoms.

Types of Dercum's Disease

Diffuse: The lipomas can be small and diffusely affect the majority of SAT; best palpated by rolling the fingers over the fat tissue. Consider initial exam in the cubital areas and medial knee.

Nodular: Lipomas can be the size of a marble, walnut, fist, or larger, localized primarily on the arms, anterior rib cage, abdomen, low back, buttocks and thighs. 

Mixed: There often is a mixed picture of diffuse and nodular lipomas. (2)

Diagnostic Criteria

Diagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Differential diagnoses include: fibromyalgia, lipedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumors. (3)  Due to the lack of awareness and knowledge about Dercum's Disease, reaching a concrete diagnosis can be very challenging, especially when no specific diagnostic procedure exists. MRls, CT scans, biopsies and symptomatic rulings can be helpful. Co-morbidities: Hypothyroidism, diabetes, autoimmune disease, fibromyalgia with the addition of adipose tissue .

Treatment

Currently, there is no cure for Dercum's Disease. Treatment consists of addressing symptoms and pain management.  

  • Anti-Inflammatory diet
  • Comfort and Mobility aids needed for activities of daily living
  • Hyperbaric Chamber or Cyclic Variations in Adaptive Conditioning™ (CVAC™)
  • Liposuction and excision of lipomas (lipomas may reoccur and
  • increase in number at excision site)
  • Manual Lymphatic Drainage (MLD)
  • NSAIDs (limited benefit)
  • Opioids (limited benefit)
  • Warm water exercise or other low impact activity
  • Weight reduction may reduce joint pain

Contraindications
Some things that may worsen Dercum's Disease symptoms are: strenuous physical therapy/exercise, repetitive motions, lack of sleep, poor diet, surgery/anesthesia, steroids, trauma and stress.

Support
Managing a chronic disease is difficult for many patients.  Many patients benefit from joining a support group, whether in person or an online community.

References:

OMIM 103200
Listed by NORD Yes
NLM MESH ID 0000274
ICD-10 ESS.2
Alternative ICD-10 G89.4
Chronic pain syndrome

Orohanet Number 36397
1 )National Organization for Rare Disorders.
https ://www.rarediseases.org/rare-d isease-information/rare-diseases/bylD/490/viewFullReport
2)http://www.lipomadoc.org/uploads/5/0/4/8/5048532/dd white_paper.pdf
3)Hansson E, Svensson H, and Brorson H Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods,
classification and management.
Orphanet J Rare Dis., 2012April 30. doi: 10.1186/1750-1172-7-23.

Printed Dercum's Disease cards are available.  Click here.