Lipedema / Lipoedema / Lipödem

Definition
Lipedema (painful fat syndrome) is a chronic disease that occurs mostly in females. It is characterized by bilateral, symmetrical fatty tissue excess, mainly in the hip region, upper and lower leg areas and combined with a tendency for leg swelling that worsens with standing. In male patients lipedema is extremely rare (1).  Lipedema is often misdiagnosed as bilateral lower extremity lymphedema or as simple obesity.

Etiology/Cause of Lipedema / Lipoedema / Lipödem

The etiology of lipedema and other types of fatty tissue enlargement is still unknown though a genetic cause is suspected.  Other possible causes of lipedema include metabolic, inflammatory, or hormonal involvement.  

Symptoms
Symptoms vary significantly by person; not all people have all symptoms
•    Symmetrical presentation, involving both legs 1
•    Pain in soft tissues at rest, and upon walking or when touched; hypersensitivity to touch 1
•    Accumulation of lipedemic fat from waist to knees or ankles, sometimes leaving a distinct fat ring above the ankle, leaving the feet unaffected 2
•    Hyper-mobility 2
•    Fat pads above, inside and below knees, and in outer region of upper thighs 1

•    Fat lobes or pads may put stress on joints causing abnormal gait and/or increased joint pain 2

•    Knee problems which may lead to gait impairment 2

•    Accumulation of lipedemic fat in the upper arms, sometimes leaving large amounts of arm fat hanging when arm is outstretched, research reports a range of 30-90% of people with lipedema are affected in their arms 2

•    Accumulation of lipedemic fat in the arms, sometimes creating a blouse effect as the distinct fat ring above the wrist forms 2
•    Loss of skin elasticity 1
•    Non-pitting edema initially; later may become lipo-lymphedema (Stage IV) 1

•    Pitting edema can happen with prolonged orthostasis and should resolve when legs are elevated 2
•    Caloric restrictive diets have little effect on lipedemic fat 1
•    Easy bruising 1
•    Negative Stemmer’s sign on feet and hands 1
•    Decreased skin temperature in the limbs 3
•    General fatigue 3
•    Sensitivity to pressure 2
•    The limb texture is rubbery 3
•    Age of onset is usually during puberty, pregnancy or menopause 1
•    Onset or exacerbation can present after surgery with general anesthesia 3
•    Feet and hands are generally unaffected before development of Lipo-Lipedema (Stage IV) 1
•    Early stages: The upper body may remain slender as the lower body enlarges and fat accumulates in the hips, thighs and legs 1
•    Later stages: lipedemic fat also may accumulate in the chest, torso, abdomen and upper extremities 3
•    Obvious lymphatic involvement is present in the later stages 2
•    Chronic pain and embarrassment may lead to depression and/or eating disorders 2, 3
•    Progressive deterioration of mobility if left untreated 1
•    Fat may become fibrotic 4
•    Low Vitamin D, iron and/or B12 4
•    Sometimes pockets of fat/fluid appear below the knee 3

1cited in many papers

2cited in some papers

3result of patient surveys

4anecdotal


Stages of Lipedema (2)
Stage I
1.    Skin is smooth
2.    Swelling increases during the day and may resolve with rest and elevation
3.    Responds well to treatment
Stage II
1.    Skin has indentations
2.    Lipomas may develop
3.    Eczema and erysipelas may be present
4.    Swelling increases during the day, with less resolution after rest and elevation
5.    May respond well to treatment
Stage III
1.    Hardened connective tissue/fibrosclerosis
2.    Swelling consistently present
3.    Large masses of skin and fat that overhang
4.    Less responsive to some treatment modalities
Stage IV
1.    Fibrosclerosis, possibly elephantiasis
2.    Swelling consistently present
3.    Larger masses of skin and fat that overhang
4.    Also known as Lipo-Lymphedema
5.    Less responsive to some treatment modalities

Differential Diagnosis 3, 7, 8, 9

Lipedema Lymphedema Obesity Lipo-lymphedema Venous Problems

Fat deposits / swelling in legs and/or arms NOT hands/feet

Fat deposits / swelling in one limb including hands/feet

Fat deposits

widespread

Fat deposits / swelling widespread in legs/arms/torso

Swelling near ankles; brownish discoloration of the legs. Minimal swelling possible.

F F/M F/M F F/M

Around hormonal shifts (puberty, pregnancy, menopause)

After surgery that affects lymphatic system, or at birth

Any age

Around hormonal shifts 

Around onset of obesity, diabetes, pregnancy, hypertension

Restricting calories ineffective

Restricting calories ineffective

Diets and weight loss strategies often effective

Restricting calories ineffective

No relation to caloric intake
Non-pitting edema Pitting edema No edema Lots of edema; some pitting; some fibrosis

Often edema, but can also occur without edema in earlier stages

Stemmer's Sign negative Stemmer's Sign positive Stemmer's Sign negative Stemmer's Sign positive or negative  
Pain in affected areas likely  No pain initially No pain  Pain in affected areas  Pain is likely

Best estimate is 11% adult women (study done in Germany) 

Low >=30% of US adults

Unknown; best guesstimate is a few percent of adult women

>30% of US adults
No history of cellulitis Possible history of cellulitis   Likely history of cellulitis Often itching +/- discoloration mistaken for cellulitis
Family history likely Family history not likely unless primary lymphedema Family history likely Family history of lipedema likely Very likely family history

 

Lipedema/Lymphedema Differentiation
To further explain lipedema, it is necessary to compare this condition to lymphedema. Lymphedema is usually asymmetrical and can be either acquired (through surgery, trauma or infection damaging the lymphatic system) or congenital (hereditary changes in the lymphatic system). The symmetrical enlargement of both lower limbs, from waist to ankles is a hallmark of lipedema.  As the swelling continues and spreads from lower extremities to other parts of the body, the swelling is likely caused by slower lymphatic flow and changes in the lymphatic vessel structure caused by pressure in the lipedematous limbs. This is known as Lipo-Lymphedema. Lipo-Lymphedema can also develop in combination with chronic venous insufficiency, and other vascular disorders.

Lipedema can be under-diagnosed due the difficulty in differentiating it from other edemas. Some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas can be found in the article "Edema: Diagnosis and Management." (3)

Diagnosis
Currently, the best way to diagnose lipedema is performing manual inspection by a trained physician. Some clinicians may be familiar with the distinction between lipedema, lymphedema and obesity. Upon palpation of the fat the healthcare professional may feel tiny pebbles or pea like nodules. As the disease advances the nodules may increase in size and in number and may form strands of nodules. Communication between patients, clinicians and physicians should be encouraged.  

A definitive blood test for lipedema does not currently exist, therefore, some doctors recommend treating conditions that may exacerbate the lipedema symptoms.  They may order tests to consider thyroid levels, markers of inflammation, vitamin D and B levels etc.  At some stages of the disease it may be possible to see evidence of lipedema or lipo-lymphedema via ultrasound, MRI, lymphangiogram and/or lymphoscintigraphy. However caution is needed due to test results that may appear ‘normal’ when testing the early stages of lipedema.  Such scans and diagnostic tools may inform the physician as to a treatment protocol.

Schmeller distinguishes lipedema from lipohypertrophy (lipo-hypertrophy) which may present with a similar body shape but without pain and edema. He postulates that one condition may lead to the other and that people may move from one state to another. (4)  More research needs to be done to determine if lipedema and lipohypertrophy are the same condition or are different from one another.

Treatment
Even though lipedema was first formally identified in the U.S. at the Mayo Clinic in 1940 (5), most physicians are not readily familiar with the disorder. There is no cure yet identified for lipedema, however, early detection and treatment can significantly reduce the debilitation of the patient.  Early weight and diet changes through nutrition and exercise may be helpful in reducing non-lipedemic fat and reducing inflammation.  This approach may possibly prevent the lower body from enlarging as much as it would if the patient were to become obese. However, even with strict diet and exercise regimens the disease may progress and further treatments may be necessary.  

While there is no proven effective treatment for lipedema, the swelling may be somewhat managed with Compression garments, certain Products and TreatmentsManual Lymphatic Drainage and/or Complete Decongestive Therapy.  Compression garments and other products can further help reduce the swelling and lessen the fluid return.  Unfortunately, these garments may not be well tolerated by all patients if the patient experiences pain.  It is important to work with a qualified provider to find the correct garments as the wrong garment can compress the initial lymphatic openings inhibiting lymph flow through the lymphatic vessels.

Liposuction as a treatment for lipedema was developed in Germany in the late 1980s (6) and is becoming more common. While it has helped some patients when done by a skilled surgeon knowledgeable about lipedema, very few physicians in the United States have the knowledge and experience of physicians in Europe. Given the fact that liposuction is a surgery and all surgeries come with risk, consulting one’s primary care physician before surgery is recommended.  Currently, different liposuction procedures used for lipedema patients are available but not all surgeries are suitable for all patients. Water Assisted Liposuction (WAL) and Tumescent Liposuction (TLA) are two procedures that are routinely and successfully being performed in Germany, the Netherlands, the UK and Austria for lipedema patients. Patients are encouraged to consult with multiple physicians who perform liposuction procedures to gain insight into the physician’s approach, outcomes and technique.

Lipedema is sometimes accompanied by co-morbidities, often secondary diseases, which complicate the patient’s health.  Articular and venous diseases, lymphedema, obesity, and psycho-social disorders are often seen with lipedema patients.  Medications for pain control, anxiety and depression are common prescriptions in this population. Patients are urged to join social support groups available on the internet and attend events.  Some patients report reduction in symptoms with certain medicines and supplements. Gastric bypass or Bariatric surgery will not reduce the lipedemic fat, though it may help ease some of the co-morbidities that may be present.

Please consult with your doctor about these options.

Research
Though it is currently somewhat recognized, treated, and researched in Europe, lipedema is under-researched in the United States. However, recent strides in the recognition of lipedema as a distinct medical condition have been successful. In October 2013, a MeSH term for lipedema was created and an ICD code proposal is currently in review.  Interest in lipedema has increased over the years as evidenced by numerous published medical research papers.  Additional research may lead to additional insurance coverage in the US.

Given that lipedema is thought to affect 11% of the adult female population (7), it is vital that more research funding be allocated and larger scale studies take place.

Coding

ICD-11 "LF14" http://apps.who.int/classifications/icd11/browse/l-m/en#!http%3A%2F%2Fid.who.int%2Ficd%2Fentity%2F1172950828

ICD-10/9 There is no clear-cut code, but some physicians use:

457.1 "lymphedema" if there is swelling component

782.3 "edema"

Q82.0 "hereditary lymphedema" http://www.icd10data.com/ICD10CM/Codes/Q00-Q99/Q80-Q89/Q82-/Q82.0

272.8 "lipomatosis" 

E65 "swelling" 

Mesh DO65134

ORPHA 77243

OMIM 614103

Synonyms
Lipalgia
Adiposalgia/Adipoalgesia
Adiposis dolorosa
Lipomatosis dolorosa of the legs
Lipohypertrophy dolorosa
Painful column leg
Painful lipedema syndrome
Lipoedema (UK and Australian spelling)
Lipödem (German spelling)
Lip (jargon)

Lipedema Patients
Lippies
Lippy Ladies
Lipese

Books

There are >20 books that mention lipedema, see the list here http://fatdisorders.org/research/books

Educational materials

eLearning from RCGP in the UK

http://www.rcgp.org.uk/courses-and-events/online-learning/ole/lipoedema-an-adipose-tissue-disorder.aspx

Therapists and some physicians

http://www.chirocredit.com/courses/index.php?catid=131&pid=20 then scroll to Lymphology 101; $18

Nursing

http://www.nursingcenter.com/lnc/CEArticle?an=00129334-201002000-00010&Journal_ID=54015&Issue_ID=965181 ; $24.95

For Physical Therapists

http://ce.todayinpt.com/course/pt07/lipedema-often-confused-with-lymphedema/ ; $19


Further Reading

There are >160 papers on lipedema listed on our site here: http://fatdisorders.org/research/lipedema-lipoedema-research-papers

 

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Bibliography
1)  Chen, S. G., Hsu, S. D., Chen, T. M., Wang, H. J. Painful fat syndrome in a male patient. Br J Plast Surg 2004; 57(3):282-286
2)  http://www.tillysmidt.nl/LIPEDEMA%20%20Englisch%20for%20Lipoedeem.htm, adapted from this site
3)  Todd, Marie, Lipoedema: presentation and management. Chronic Oedema, 2010; 10-16
4)  Schmeller W, Meier-Vollrath I, Chapter 7 Lipedema, http://www.hanse-klinik.com/englisch/Lipoedema.pdf 
5)  Allen E V, Hines E A, Lipoedema of the legs. A syndrome characterized by fat legs and edema. Proc Staff. Meet. Mayo Clinic 1940; 15:184
6)  C to provide
7)  Fife CE, Maus EA, Carter MJ, 2010 Lipedema: A Frequently Misdiagnoed and Misunderstood Fatty Deposition Syndrome. ADV SKIN WOUND CARE 2010;23:81-92; quiz 93-4. reported "However, in an unpublished epidemiological study conducted in 2001, Foeldi and Foeldi [28] claimed that lipedema is present in 11% of the female population". [28] corresponds to "Foeldi E, Foeldi M. Lipedema. In: Foeldi M, Foeldi E, eds. Foeldi’s Textbook of Lymphology. 2nd ed. Munich, Germany: Elsevier; 2006:417-27.

8) Trayes KP, Studdiford JS, Pickle S, Tully AS  “Edema: Diagnosis and Management” Am Fam Physican 2013:88(2):102-110

http://www.ncbi.nlm.nih.gov/pubmed/23939641 

9) Herbst, Karen L “Rare adipose disorders (RADs) masquerading as obesity” Acta Pharmacol Sin. 2012 Feb;33(2):155-72. doi: 10.1038/aps.2011.153.

http://www.ncbi.nlm.nih.gov/pubmed/22301856