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.
DIFFERENTIATIONPRIMARY LYMPHEDEMASECONDARY LYMPHEDEMALIPEDEMALIPO-LYMPHEDEMA (lipedema with secondary lymphedema)OBESITYVENOUS INSUFFICIENCY
GenderFemale > MaleFemale > Male (most common cause of secondary lymphedema in the Western world is cancer therapeutics )Almost exclusively female; seen only in males with feminizing endocrine statusAlmost exclusively female; seen only in males with feminizing endocrine statusFemale and MaleFemale > Male
OnsetCan occur in congenital, pubertal and adult onset formsDepends on etiology. Can be weeks, months or even years delayed from inciting event (i.e. surgery, infection, trauma, etc.)Most frequently with the menarcheAs result of longstanding later stage lipedema, often combined with obesityGenetic factors or acquiredAdult with onset 30's but seen across all ages
DevelopmentUsually starts distal and progresses toward proximalUsually starts distal and progresses toward proximalGradual, bilateral lower extremities without foot involvementBilateral lower extremities with or without foot edemaGradual, affecting entire bodyGradual, lower leg(s) affected with dermatitis and ulcers but varicose veins seen thruout entire leg, typically sparing the feet, unless there is secondary lymphedema
ExtentMay 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 areaFrom the iliac crest to the ankle; no involvement of the dorsum of the feetFrom the iliac crest to the ankle; with involvement of the dorsum of the feetWhole bodySwelling usually progresses from distal to proximal and spares the feet
Stemmer’s signPositiveMay be positiveNegativeNegative or positiveNegativeNegative
HypermobilityNoNoYesPossibleNoNo
DistributionUnilateral or bilateral, if bilateral asymmetricUnilateral or bilateral, if bilateral usually asymmetricSymmetric distribution of adipose tissue from the hips and ankles, the feet are not involved; disproportion between upper and lower body unless combined with obesitySymmetric distribution of adipose tissue from the hips and ankles and involvment of the feet; disproportion between upper and lower body unless combined with obesityUsually symmetricUnilateral or bilateral, if bilateral, usually fairly symmetric
Pain / hypersensitivity of affected tissueNoWith onset there can be pain, as lymphedema progresses there is little or no painYesYesNo (although knee pain is common due to arthritis)Common
Skin temperatureNormalNormalNormal or slightly decreasedNormal or slightly decreasedNormal or slightly decreasedNormal or slightly increased
Skin colorNormalNormal or sometimes pinkNormalNormalNormalReddish brown discoloration (hemosiderin staining); dependent rubor
BruisingNormalNormalCommon, even after minor traumaCommon, even after minor traumaNormalCommon, even after minor trauma
Tissue consistencyInitially soft, then harder because of progressive lymphostatic fibrosclerosisInitially soft, then harder because of progressive lymphostatic fibrosclerosisSoftInitially soft, may become indurated because of progressive lymphostatic fibrosclerosisSoftLipo-dermato-sclerosis with or without ulcerations, severe dermatitis will be firm and indurated/woody
EdemaPitting in earlier stages, later fibrosclerosisPitting in earlier stages, later fibrosclerosisMinimal or no pitting edema of the lower legs, only after prolonged orthostasisPitting edema in the areas affected by lymphedema (lower legs and feet)No pittingPitting edema may occur
Dorsum of the feetEdema in most casesEdema in most casesNo edemaEdemaNo edemaUsually spared
Hyperkeratosis (abnormal thickening of the outer layer of the skin)In severe casesIn severe casesNoPossibleNoAdvanced stages have thick indurated woody brawny dermatitis with lipodermatosclerosis and possible ulceration
CellulitisCommonCommonNoIn advanced stagesNot obesity relatedPossible
Influence of positioning on edemaDecreases in stage 1 and 2Decreases in stage 1 and 2Only decreases the orthostatic edemaDecreasesDoes not applyDecreases with elevation
HereditaryOnly 2% are familialDoes not applyMay be familialMay be familialMay be familialMay be familial
Number affected1/100,000Approximatley 5-8% of women undergoing sentinel LN biopsy for breast cancer, and up to 40-50% of patients having radiation/lymph node dissections11% women (according to Földi, Textbook on Lymphology, 3rd edition)Unknown69% of adult population is obese and overweight in United States (according to CDC)25-40% of adult population
LymphoscintigraphyAbnormalAbnormalNormal or sometimes increased uptakeAbnormalNormal unless accompanied by lymphedemaNormal or increased uptake unless accompanied by lymphedema
Lymphangiography w/Indocyanine greenAbnormalAbnormalNormal or slightly increasedAbnormalNormal unless accompanied by lymphedemaNormal or increased uptake unless accompanied by lymphedema
ICD 10 CodeI89.0 Lymphedema, not elsewhere classifiedI89.0 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