Differential Diagnosis

Lipedema is just one of several existing fat disorders, and is often misdiagnosed because it is difficult to differentiate from related conditions. While there are similarities between these disorders, conditions such as lipedema, lipolymphedema, primary lymphedema, obesity, and venous insufficiency are all distinct, and their differences must be understood in order to make an accurate diagnosis.

NOTE: ICD10 codes for lipedema are now included in Germany (as of early January 2017). The codes are listed below and include Stage 1, Stage 2, Stage 3, and Other. Check for ICD10 inclusion for your country (ICD10 codes do not as of yet include lipedema in the USA).

DIFFERENTIATION LIPEDEMA LIPO-LYMPHEDEMA (lipedema with secondary lymphedema) PRIMARY LYMPHEDEMA SECONDARY LYMPHEDEMA OBESITY VENOUS INSUFFICIENCY
Gender Almost exclusively female; seen only in males with feminizing endocrine status Almost exclusively female; seen only in males with feminizing endocrine status Female > Male Female > Male (most common cause of secondary lymphedema in the Western world is cancer therapeutics ) Female and Male Female > Male
Onset Most frequently with the menarche As result of longstanding later stage lipedema, often combined with obesity Can occur in congenital, pubertal and adult onset forms Depends on etiology. Can be weeks, months or even years delayed from inciting event (i.e. surgery, infection, trauma, etc.) Genetic factors or acquired Adult with onset 30's but seen across all ages
Development Gradual, bilateral lower extremities without foot involvement Bilateral lower extremities with or without foot edema Usually starts distal and progresses toward proximal Usually starts distal and progresses toward proximal Gradual, affecting entire body Gradual, lower leg(s) affected with dermatitis and ulcers but varicose veins seen thruout entire leg, typically sparing the feet, unless there is secondary lymphedema
Extent From the iliac crest to the ankle; no involvement of the dorsum of the feet From the iliac crest to the ankle; with involvement of the dorsum of the feet May involve the whole leg and foot or just the distal leg and foot. Can affect only a portion of the extremity and does not need to be most dependent area Whole body Swelling usually progresses from distal to proximal and spares the feet
Stemmer’s sign Negative Negative or positive Positive May be positive Negative Negative
Hypermobility Yes Possible No No No No
Distribution Symmetric distribution of adipose tissue from the hips and ankles, the feet are not involved; disproportion between upper and lower body unless combined with obesity Symmetric distribution of adipose tissue from the hips and ankles and involvment of the feet; disproportion between upper and lower body unless combined with obesity Unilateral or bilateral, if bilateral asymmetric Unilateral or bilateral, if bilateral usually asymmetric Usually symmetric Unilateral or bilateral, if bilateral, usually fairly symmetric
Pain/hypersensitivity of affected tissue Yes Yes No With onset there can be pain, as lymphedema progresses there is little or no pain No (although knee pain is common due to arthritis) Common
Skin temperature Normal or slightly decreased Normal or slightly decreased Normal Normal Normal or slightly decreased Normal or slightly increased
Skin color Normal Normal Normal Normal or sometimes pink Normal Reddish brown discoloration (hemosiderin staining); dependent rubor
Bruising Common, even after minor trauma Common, even after minor trauma Normal Normal Normal Common, even after minor trauma
Tissue consistency Soft Initially soft, may become indurated because of progressive lymphostatic fibrosclerosis Initially soft, then harder because of progressive lymphostatic fibrosclerosis Initially soft, then harder because of progressive lymphostatic fibrosclerosis Soft Lipo-dermato-sclerosis with or without ulcerations, severe dermatitis will be firm and indurated/woody
Edema Minimal or no pitting edema of the lower legs, only after prolonged orthostasis Pitting edema in the areas affected by lymphedema (lower legs and feet) Pitting in earlier stages, later fibrosclerosis Pitting in earlier stages, later fibrosclerosis No pitting Pitting edema may occur
Dorsum of the feet No edema Edema Edema in most cases Edema in most cases No edema Usually spared
Hyperkeratosis (abnormal thickening of the outer layer of the skin) No Possible In severe cases In severe cases No Advanced stages have thick indurated woody brawny dermatitis with lipodermatosclerosis and possible ulceration
Cellulitis No In advanced stages Common Common Not obesity related Possible
Influence of positioning on edema Only decreases the orthostatic edema Decreases Decreases in stage 1 and 2 Decreases in stage 1 and 2 Does not apply Decreases with elevation
Hereditary May be familial May be familial Only 2% are familial Does not apply May be familial May be familial
Number affected 11% women (according to Földi, Textbook on Lymphology, 3rd edition) Unknown 1/100,000 Approximatley 5-8% of women undergoing sentinel LN biopsy for breast cancer, and up to 40-50% of patients having radiation/lymph node dissections 69% of adult population is obese and overweight in United States (according to CDC) 25-40% of adult population
Lymphoscintigraphy Normal or sometimes increased uptake Abnormal Abnormal Abnormal Normal unless accompanied by lymphedema Normal or increased uptake unless accompanied by lymphedema
Lymphangiography w/Indocyanine green Normal or slightly increased Abnormal Abnormal Abnormal Normal unless accompanied by lymphedema Normal or increased uptake unless accompanied by lymphedema
ICD 10 Code

R60.9 Lipoedema

Q82.0 Familial Hereditary Edema

German ICD10 codes for lipoedema

E88.20 Lipoedema, Stage 1

E88.21 Lipoedema, Stage 2

E88.22 Lipoedema, Stage 3

E88.28 Other or unspecified lipoedema

R60.9 Lipoedema

I89.0 Lymphedema, not elsewhere classified

I89.0 Lymphedema, not elsewhere classified I89.0 Lymphedema, not elsewhere classified

I97.2 Postmastectomy lymphedema syndrome

E66.9 Obesity, unspecified

E66.8 Other obesity

I87.2 Venous insufficiency (chronic) (peripheral)
Developers: Mark L Smith, MD, FACS; Guenter Klose, MLD/CDT; Professor Etelka Földi, MD; Stanley Rockson, MD; Jennifer Svahn, MD, FACS; Kimberly Gudzune, MD, MPH; Matthew Carmody, MD; Erez Dayan, MD; & Catherine Seo, PhD; Copy Editor: Beatrice Sussman

 

Lymphedema: If lipedema progresses, patients can develop secondary lymphedema, a condition characterized by fluid retention and significant swelling. The two conditions together are known as lipolymphedema. This condition appears as patients progress beyond Stage 3 lipedema into Stage 4 lipolymphededema.

Venous Insufficiency: Venous insufficiency occurs when the valves in the veins that keep blood flowing in one direction are damaged or weakened, and the veins cannot properly pump blood back to the heart. The condition usually occurs in the legs and is fairly common in lipedema patients. Patients with venous insufficiency may have varicose veins, feel heaviness or pain in their legs, and experience swelling or redness.

Pain and Loss of Mobility: Lipedema fat can be very painful, and the condition can worsen if not kept in check through a healthy lifestyle. If lipedema continues to advance, patients can become progressively less mobile. Pain and immobility may lead to obesity, which exacerbates lipedema and causes increased risk for venous insufficiency and further swelling.

Fibrosis: Fibrosis is a condition that occurs when tissues of the body have been damaged or stressed in some way. In many lipolymphedema patients, swelling in their legs from the lymphedema causes hard connective tissue to form. Fibrosis is painful and inhibits proper circulation of lymph fluid through the extremities.

About The Friedman Center

The Friedman Center for Lymphedema Research & Treatment at Northwell Health in New York was founded in 2013. Its focus has been on offering solutions for patients with lymphedema, a debilitating condition that occurs in many patients after cancer treatment.

The Friedman Center’s scope of care now includes treatment for lipedema, a condition that can also result in lymphedema. To that end, the Friedman Center has created the Lipedema Project, a comprehensive program to increase awareness and provide education, research and treatment for lipedema. The Friedman Center is proud to sponsor the lipedema documentary, The Disease They Call FAT, the 1st International Symposium on Lipedema, the Lipedema Think Tank, the Lipedema Solutions Forum for women with lipedema in April 2015 and this online CME eduring materials course for physicians.

Lipoedema Lymphedema