Skin may be smooth and soft, but homogenous increase in underlying subcutaneous tissue with enlarged underlying hypodermis with cool skin in certain areas due to functional vascular imbalance

Skin may be indented and irregular with palpable pearl-sized nodules (“peau d'orange”) appearance

It is characterised by tender subcutaneous nodules, folds and divots over deforming, larger fat masses with bulging protrusion of fat mainly at inner thighs and knees and is frequently associated with functional limitations

The development of concomitant lymphedema defines stage 4. It is usually the result of a long-term evolution of the disease from subclinical to clinically manifested lymphedema, thus representing a more advanced stage in most cases. There is a pathognomonic positive stemmer sign which may be accompanied by other common features of advanced lymphedema, such as cellulitis or papillomatosis.