Table 3.13-1. Classification and causes of lymphedema

Primary lymphedema

Secondary or acquired lymphedema

– Lymphedema congenita presenting at birth or during infancy

– Lymphedema praecox, the most common type of primary lymphedema presenting around puberty and affecting predominantly legs

– Lymphedema tarda presenting in adulthood, usually at the age of 35 years

– Infections, particularly parasitic (eg, filariasis)

– Recurrent skin infections (eg, cellulitis)

– Post surgical treatment (eg, following mastectomy or lymph node dissection in breast cancer [Figure 1] or in melanoma)

– Post radiotherapy for cancer

– Related to malignancy due to obstruction of lymphatic vessels by neoplasm (eg, ovarian and peritoneal cancers) or due to extensive involvement of lymph nodes by malignancy (lymphoma)

– Lymphedema due to extensive involvement of lymph nodes by granulomatous process (eg, sarcoidosis)

– Posttraumatic lymphedema

– Postinflammatory lymphedema (complication of arthritis, vasculitis, or lymphadenitis)

– Combined venous and lymphatic edema in patients with chronic venous insufficiency

– Idiopathic lymphedema

– Lymphedema associated with morbid obesity