Table 1

Stage I

A."Latent" lymphedema, without clinical evidence of edema, but with impaired lymph transport capacity ( provable by lymphoscintigraphy) and with initial immuno-histochemical alterations of lymph nodes, lymph vessels and extracellular matrix.

B.“Initial” lymphedema, totally or partially decreasing by rest and draining position, with worsening impairment of lymph transport capacity and of immuno-histochemical alterations of lymph collectors, nodes and extracellular matrix.

Stage II

A. “Increasing” lymphedema, with vanishing lymph transport capacity, relapsing lymphangitic attacks, fibroindurative skin changes, and developing disability.

B. "Column shaped" limb fibrolymphedema, with lymphostatic skin changes, suppressed lymph transport capacity and worsening disability.

Stage III

A. Properly called “elephantiasis”, with scleroindurative pachydermitis, papillomatous lymphostatic verrucosis, no lymph transport capacity and life-threatening disability.

B. “Extreme elephantiasis” with total disability.