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 Unspecified Edema, Lipoedema Q82.0 Familial Hereditary Edema German ICD10 codes for lipoedemaE88.20 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
| 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 Unspecified Edema, Lipoedema Q82.0 Familial Hereditary Edema German ICD10 codes for lipoedemaE88.20 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.
