<<Dercum's Disease>>

What is Dercum’s Disease (DD)? 1,2,4

Type III Dercum's Disease. Image retrieved from http://www.edoj.org.eg/vol008/0802/008/01.htm
Type III Dercum's Disease. Image retrieved from http://www.edoj.org.eg/vol008/0802/008/01.htm

Dercum's disease (DD) is an extremely rare disorder characterized by multiple, painful growths consisting of fatty tissue (lipomas)

These painful fat deposits can be anywhere in the body, with a focus on arms, neck, chest, low back, abdomen, legs and groin.

 

Other names indlude:

    Adiposis Dolorosa

    Fatty or Adipose Tissue Rheumatism

    Lipomatosis Dolorosa Morbus Dercum’s

    Adiposalgia

    Lipomatosis Dolorosa

    Juxta-Articular Adiposis Dolorosa

 

What are the causes of Dercum’s Disease?1,2,4

The causes of the disease are unknown, however, some research indicates that it might be linked with metabolic or autoimmune components.

It is reported that Dercum’s Disease runs in families. It is also thought to have a sex-specific influence, as it affects 20 times more women (mainly between the ages of 45 and 60) than men, especially women who are overweight or postmenopausal. It can also occur in children. 

It might also be related to nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma (although these are not confirmed).

What are the signs and symptoms of Dercum’s Disease? 1,3,5

 

Physical symptoms: 

         Painful lipomas, ranging in size from rice grains to a fist or larger

         Pain may be spontaneous, sporadic or upon palpation

         Pain in fat deposits for at least three months

         Non-pitting edema in subcutaneous fat

         Vascular involvement, angiolipomas

         Multiple cherry angiomas and petechiae

         Easy bruising

         Flushing

         GERD, gastro-esophageal reflux disease

         Irritable bowel syndrome, bloating, and abdominal pain

         Early satiety

         Weight gain in most cases

         Fatigue, increased by activities of daily living and exercise

         Joint pain and/or stiffness, increased in areas of fat deposits

         Muscle pain and stiffness, especially upon wakening or the day after physical activity

         Migraines

         Shortness of breath

         Tachycardia, rapid heart palpitations to supraventricular tachycardia requiring beta-blockers

         Heavy or prolonged menstrual bleeding

         Tender nodules in the vaginal vault and enlarged, swollen, and tender labia in women

         Hematuria (blood in the urine) of unknown cause

         Symptoms may progress to a point of immobility

 

Non-physical symptoms: 

         Memory difficulties/impairment

         Difficulty forming thoughts, “brain fog”

         Difficulty expressing thoughts, problems finding words 

         Depression and anxiety

         Sleep disturbance, insomnia

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

 

Types of Dercum’s Disease 1

Type I, juxta-articular (around the joint): Painful folds or nodular fat on the inside of the knees and/or on the hips; in rare cases only evident in the upper-arm

Type II, diffuse, generalized type: Widespread pain from fatty tissue found anywhere from head to the soles of the feet

Type III, nodular type: Intense pain in and around multiple “lipomas”, sometimes in the absence of obesity

 


Treatment options for Dercum’s Disease 1,2,3

Currently, there is NO cure for Dercum’s Disease.

The goals of treatment are to address symptoms and manage pain by using the following modalities.

    Analgesics (opiates and NSAIDs); may help some patients, and some patients report limited relief

    Weight reduction; helps to relieve some of the joint pain

    Surgical excision of lipomas; may affect patient comfort however, lipomas have been known to reoccur and increase in number at the same site or nearby location

    Manual Lymph Drainage (MLD); see below for details

 

How can MLD help manage Dercum’s Disease? 2,3,6,7

It is reported that Dercum’s Disease responds to MLD.

 

The lymphatic system is a drainage system that removes substances from the interstitium (space between tissues). As Dercum’s Disease progresses, the skin changes and becomes thick or thin, fibrotic, loses elasticity, and/or develops varicosities, etc. As a result, the lymph vessels become dilated and lymphatic flow is compromised, which leads to swelling. Dercum’s Disease is a pre-lymphedema condition.

 

Manual Lymph Drainage (MLD) is a type of massage, targeting the lymphatic system and lymphatic flow. The treatment is extremely gentle with no deep pressure applied.

MLD helps maintain healthy tissue integrity by bringing fresh fluids to, and removing metabolic wastes and excess fluids away from the area. This is achieved by increasing the flow of lymphatic fluids and redirecting and/or removing excess lymph from the affected area(s), in order to reduce swelling.

 

The repetitive motion of MLD will block pain signals from travelling to the brain, thus effectively reducing pain.

 

MLD also helps calm down the sympathetic nervous system and triggers pleasure reflexes, giving the patient a feeling of well-being. Many people find MLD very soothing. 

 

What should I expect during MLD treatment for Dercum’s Disease? 3,6,7

Your therapist first assesses your overall condition, your comfort level, and your pain level.

 

Based on the findings, individual treatment plans are carefully developed. 

 

Manipulation is very gentle, yet removes great amounts of excess fluids from the affected areas. If pain is severe, MLD would be done around the lipomas, avoiding direct contact with the painful areas.

 

When necessary, treatment might include the abdomen to help clear any obstructions, which will help lymphatic fluid return from the lower extremities and genitals to the circulatory system.

 

Some people with Dercum’s Disease may experience worsening of their symptoms after MLD, such as gastrointestinal distress and pain. This is a part of the healing reactions and should subside in a matter of days.

 

The number of treatments and frequency vary depending on the severity. However, it is often done more frequently for the first few weeks (intensive phase), then moves on to the maintenance phase.

 

Progress assessment is performed as needed. 

 

Regardless of the severity, Dercum’s Disease has no cure. Life-long maintenance MLD treatments are recommended, which prevent worsening of the condition.

 

When the condition is severe, it may require more frequent treatments.

 

If you have more swelling and it manifests as Lymphedema, compression therapy (compression bandaging and/or compression garments) would be suggested.

 

If you or a loved one are experiencing Dercum’s Disease, please do not hesitate to contact us to find out if/how MLD can help you get back to enjoying your daily activities.

 

Important Note

Karen. L. Herbst, PhD, MD, world-renowned expert in this disease, requires the therapist who provides MLD to complete all courses for MLD for Lymphedema to treat Dercum's Disease.3 Make sure that your therapist has completed all courses and holds a certificate for Combined Decongestive Therapy (CDT). This ensures optimal and safe treatment.

 

Akari Yokokawa, RMT/CDT, has completed all MLD and CDT training with Vodder School International and is able to provide the gold standard treatment protocol for lymphedema. She has been attending bi-annual recertification courses and other lymphedema-related workshops and courses to update/refresh her skills and knowledge about MLD and lymphedema since she obtained a CDT certificate.

She is passionate about educating patients about life-style changes and self-care regimens. Her treatments and education often include self-lymph drainage, gentle lymphatic exercise, skin care, and other modalities to help patients better maintain their condition based on their treatment goals.

 

If you have questions or would like to book an appointment with Akari, please visit here.

 

Rererences;

1) http://fatdisorders.org/fat-disorders/dercums-description

2) http://www.lipomadoc.org/dercums-disease.html 

3) Karen, L, Herbst PhD, MD. Manual Lymph Drainage for Dercum’s Diesase. www.lipomadoc.org

4) https://rarediseases.org/rare-diseases/dercums-disease/

5) Karen, L. Herbst, PhD, MD  Dercum’s Disease White Paper (information for Healthcare Providers). 

6) Hildegard Wittlinger et al. Dr. Vodder’s Manual Lymph Drainage A Practical Guide.  2011. Thieme New York 

7) Renato Kasseroller. Compendium of Dr. Vodder’s Manual Lymph Drainage. 1998. HAUG