b'Staging of LipedemaLipedema is classified in stages by observationalSTAGE III: Increased lipedema tissue more fibroticcharacteristics in the extremities 21 : in texture with numerous large subdermal nodulesSTAGE I: Smooth skin; homogenous increase inand overhanging lobules of tissue. subcutaneous tissue. STAGE IV: A pronounced increase in circumference STAGE II: Irregular skin surface, nodular changes of with loose skin/tissue.the subcutaneous tissue.Figure 3. Stages of LipedemaDiagnosis: A General Overview treatment measurements of the upper limbs for aThroughout the evaluation of the patient, the followingmore accurate baseline, as weight fluctuations differential diagnoses should be considered in order throughout treatment can obscure lymphedema-driven to conduct a more focused assessment: cancer-limb volume changes that occur post-treatment.related or non-cancer-related lymphedema, lipedema,Circumferential measurements using a tape measurecongenital vascular anomalies with discernmentis a common assessment method, with limb volumes between vascular, lymphatic, and combined disorders,easily calculated from circumference measures by systemic lymphatic disorders, protein-losingusing the truncated cone volume formula 22 .enteropathy, lymphangiectasias, lymphaticovenousMeasurements are completed at standard distances disease, pediatric lymphatic diseases, filariasis,(typically 4 centimeters) apart along the limb using a podoconiosis, and complex lymphatic anomalies tape measure. Baseline measurement (for example, such as Gorhams Stout Disease (GSD), before cancer treatment) or the unaffected Generalized Lymphatic Anomaly (GLA), Kaposiformcontralateral limb is used as a reference, with volume Lymphangiomatosis (KLA), Central Conductingdifferences of 200cc or 510% seen as diagnostic.Lymphatic Anomaly (CCLA). See Figure 4 (on next page) for a diagnosis algorithm. However, one must be aware that in complex vascular anomalies such as Klippel-Trenaunay Syndrome (KTS), ASSESSMENT TOOLS USED IN Proteus syndrome, and PIK3CA-related overgrowth THE DIAGNOSIS OF LYMPHEDEMA spectrum (PROS), individuals may present with visible AND LIPEDEMA overgrowth of various body parts in addition to having lymphedema, so the diagnostic criteria would not Author: Jasmine Zhang, MD, CLT, FAAPMR apply here. In addition, these measurements do not In addition to a comprehensive history and physicalconsider changes in fat or muscle mass. exam, there are additional tools that can be utilized Volume measurements can also be performed via a to further measure lymphedema (LE) and lipedema.perometer, a machine that uses photosensors and These tools can be used to assist in the diagnosis andlight-emitting diodes to scan a limb. While it has good monitoring of disease progression and response toreliability, these machines are expensive and thus not treatment interventions. Additionally, these tools canreadily available in non-research settings 23 . Water be used in surveillance programs; for example, prior todisplacement is another tool in which the limb under breast cancer treatment, it is optimal to obtain pre-| Standardized Approach for the Diagnosis and Management of Lymphedema (LE) and Lymphatic Diseases (LD)|11'