Criteria

Clinical classification based on disease stage defined by the International Society of Lymphology (ISL)[47]

  • Stage 0: latent or subclinical condition; swelling is not evident despite impaired lymph transport; may exist months or years before edema is clinically apparent.

  • Stage 1: early accumulation of protein-rich fluid that improves with limb elevation; pitting may occur.

  • Stage 2: pitting is manifest; limb elevation rarely reduces tissue swelling; in later stages excess subcutaneous fat and fibrosis develop and the limb may not pit.

  • Stage 3: lymphostatic fibrosclerosis (previously known as lymphostatic elephantiasis) with absent pitting; trophic skin changes, such as acanthosis, fat deposits, and warty overgrowths develop.

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