Differential Diagnosis
Lymphedema can be challenging to diagnose as the symptoms experienced can mimic that of other conditions. It is important to understand the similarities and differences in some closely related conditions, in order to achieve proper diagnosis and treatment.
| DIFFERENTIATION | PRIMARY LYMPHEDEMA | SECONDARY LYMPHEDEMA | LIPEDEMA | LIPO-LYMPHEDEMA (lipedema with secondary lymphedema) | OBESITY | VENOUS INSUFFICIENCY |
|---|---|---|---|---|---|---|
| Gender | Female > Male | Female > Male (most common cause of secondary lymphedema in the Western world is cancer therapeutics ) | Almost exclusively female; seen only in males with feminizing endocrine status | Almost exclusively female; seen only in males with feminizing endocrine status | Female and Male | Female > Male |
| Onset | 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.) | Most frequently with the menarche | As result of longstanding later stage lipedema, often combined with obesity | Genetic factors or acquired | Adult with onset 30's but seen across all ages |
| Development | Usually starts distal and progresses toward proximal | Usually starts distal and progresses toward proximal | Gradual, bilateral lower extremities without foot involvement | Bilateral lower extremities with or without foot edema | 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 | 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 | 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 | Whole body | Swelling usually progresses from distal to proximal and spares the feet |
| Stemmer’s sign | Positive | May be positive | Negative | Negative or positive | Negative | Negative |
| Hypermobility | No | No | Yes | Possible | No | No |
| Distribution | Unilateral or bilateral, if bilateral asymmetric | Unilateral or bilateral, if bilateral usually asymmetric | 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 | Usually symmetric | Unilateral or bilateral, if bilateral, usually fairly symmetric |
| Pain / hypersensitivity of affected tissue | No | With onset there can be pain, as lymphedema progresses there is little or no pain | Yes | Yes | No (although knee pain is common due to arthritis) | Common |
| Skin temperature | Normal | Normal | Normal or slightly decreased | Normal or slightly decreased | Normal or slightly decreased | Normal or slightly increased |
| Skin color | Normal | Normal or sometimes pink | Normal | Normal | Normal | Reddish brown discoloration (hemosiderin staining); dependent rubor |
| Bruising | Normal | Normal | Common, even after minor trauma | Common, even after minor trauma | Normal | Common, even after minor trauma |
| Tissue consistency | Initially soft, then harder because of progressive lymphostatic fibrosclerosis | Initially soft, then harder because of progressive lymphostatic fibrosclerosis | Soft | Initially soft, may become indurated because of progressive lymphostatic fibrosclerosis | Soft | Lipo-dermato-sclerosis with or without ulcerations, severe dermatitis will be firm and indurated/woody |
| Edema | Pitting in earlier stages, later fibrosclerosis | Pitting in earlier stages, later fibrosclerosis | 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) | No pitting | Pitting edema may occur |
| Dorsum of the feet | Edema in most cases | Edema in most cases | No edema | Edema | No edema | Usually spared |
| Hyperkeratosis (abnormal thickening of the outer layer of the skin) | In severe cases | In severe cases | No | Possible | No | Advanced stages have thick indurated woody brawny dermatitis with lipodermatosclerosis and possible ulceration |
| Cellulitis | Common | Common | No | In advanced stages | Not obesity related | Possible |
| Influence of positioning on edema | Decreases in stage 1 and 2 | Decreases in stage 1 and 2 | Only decreases the orthostatic edema | Decreases | Does not apply | Decreases with elevation |
| Hereditary | Only 2% are familial | Does not apply | May be familial | May be familial | May be familial | May be familial |
| Number affected | 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 | 11% women (according to Földi, Textbook on Lymphology, 3rd edition) | Unknown | 69% of adult population is obese and overweight in United States (according to CDC) | 25-40% of adult population |
| Lymphoscintigraphy | Abnormal | Abnormal | Normal or sometimes increased uptake | Abnormal | Normal unless accompanied by lymphedema | Normal or increased uptake unless accompanied by lymphedema |
| Lymphangiography w/Indocyanine green | Abnormal | Abnormal | Normal or slightly increased | Abnormal | Normal unless accompanied by lymphedema | Normal or increased uptake unless accompanied by lymphedema |
| ICD 10 Code | I89.0 Lymphedema, not elsewhere classified | I89.0 Lymphedema, not elsewhere classified I97.2 Postmastectomy lymphedema syndrome | 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 | 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
Edited from copy found at lipedemaproject.org