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Lymphedema: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Lymphedema is swelling of a limb due to hypoplasia of the lymphatic vessels (primary lymphedema) or their obstruction or destruction (secondary). Symptoms of lymphedema include brown skin color and firm (no dent when pressed with a finger) swelling of one or more limbs. Diagnosis is by physical examination. Treatment of lymphedema includes exercise, compression stockings, massage, and (sometimes) surgery. A cure is not usually achieved, but treatment may reduce symptoms and slow or stop the progression of the disease. Patients are at risk of developing panniculitis, lymphangitis, and (rarely) lymphangiosarcoma.
Lymphedema can be primary (arising from lymphatic vessel hypoplasia) or secondary (developing as a result of obstruction or destruction of lymphatic vessels).
[ Primary lymphedema
Primary lymphedemas are hereditary and rare. They vary in phenotypic manifestations and the age at which the first manifestations occur.
Congenital lymphedema appears before the age of 2 years and is due to hypoplasia or aplasia of the lymphatic vessels. Milroy disease is an autosomal dominant inherited form of congenital lymphedema attributed to VEGF3 mutations and sometimes associated with cholestatic jaundice and edema or diarrhea due to protein-losing enteropathy caused by lymphatic congestion in the intestinal tissues.
Most lymphedemas appear in the age range from 2 to 35 years, in women the onset of menstruation or pregnancy is typical. Meige's disease is an autosomal dominant hereditary form of early lymphedema, attributed to mutations in the transcription factor gene (F0XC2) and associated with the presence of a second row of eyelashes (distichiasis), cleft palate and swelling of the leg, arm and sometimes face.
The onset of late lymphedema occurs after age 35. There are familial and sporadic forms, the genetic aspects are unknown. Clinical findings are similar to those of early lymphedema, but may be less pronounced.
Lymphedema occurs in a variety of genetic syndromes, including Turner syndrome, yellow nail syndrome (characterized by pleural effusion and yellow nails), and Hennekam syndrome, a rare congenital syndrome characterized by intestinal (or other localized) lymphangiectasias, facial cranial anomalies, and mental retardation.
Secondary lymphedema
Secondary lymphedema is a much more common pathology than primary lymphedema. Its causes are usually surgery (especially lymph node removal, typical in breast cancer operations), radiation therapy (especially to the axillary or inguinal region), trauma, obstruction of a lymphatic vessel by a tumor, and (in developing countries) lymphatic filariasis. Moderate lymphedema may also develop when lymph leaks into interstitial structures in patients with chronic venous insufficiency.
Symptoms of secondary lymphedema include painful discomfort and a feeling of heaviness or fullness in the affected limb.
The cardinal sign is soft tissue edema, which is divided into three stages.
- In stage I, after pressing, pits remain in the edema area, and the damaged area returns to normal by morning.
- In stage II, there are no pits left after finger pressure, and chronic inflammation of the soft tissues causes early fibrosis.
- In stage III, the skin over the affected area becomes brown and the swelling is irreversible, largely due to fibrosis of the soft tissues.
The swelling is usually unilateral and may increase in warm weather, before menstrual bleeding, and after prolonged immobilization of the limb. The process may affect any part of the limb (isolated proximal or distal lymphedema) or the entire limb. Limitations in range of motion may occur if the swelling is localized around a joint. Disability and emotional stress may be significant, especially if the lymphedema has resulted from medical or surgical treatment.
Common skin changes include hyperkeratosis, hyperpigmentation, warts, papillomas and mycoses.
Lymphangitis most often develops when bacteria enter through cracks in the skin between the toes as a result of mycosis or through cuts on the hands. Lymphangitis is almost always streptococcal and causes erysipelas, sometimes staphylococci are found.
The affected limb becomes hyperemic and hot, red streaks may extend proximal to the lesion. Lymph node enlargement may occur. Sometimes cracks appear on the skin.
Diagnosis of lymphedema
The diagnosis is usually obvious on physical examination. Additional tests are indicated if secondary lymphedema is suspected. CT and MRI can identify areas of lymphatic vessel obstruction. Radionuclide lymphoscintigraphy can detect lymphatic vessel hypoplasia or decreased lymphatic flow velocity. Negative changes can be detected by measuring limb circumference, determining the volume of fluid displaced by immersion, or using skin or soft-tissue tonometry; these tests have not been validated. In developing countries, testing for lymphatic filariasis is needed.
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Prognosis and treatment of lymphedema
Complete recovery is not typical if lymphedema develops. Optimal treatment and (possibly) preventive measures can somewhat alleviate symptoms and slow or stop the progression of the disease. Occasionally, long-standing lymphedema leads to lymphangiosarcoma (Stewart-Treves syndrome), usually in women after mastectomy and in patients with filariasis.
Treatment of primary lymphedema may include soft tissue excision and lymphatic vessel reconstruction if this results in a significant improvement in quality of life.
Treatment of secondary lymphedema involves possible elimination of its cause. Several techniques can be used to eliminate the symptoms of lymphedema in order to remove excess fluid from the tissues (complex decongestant therapy). These include manual lymphatic drainage, in which the limb is elevated and the blood is directed toward the heart with pressing movements; the pressure gradient is maintained by bandaging the limb, performing exercises, massaging the limb, including intermittent pneumatic compression. Sometimes surgical resection of soft tissue, creation of additional anastomoses of lymphatic vessels, and formation of drainage channels are used, but the methods have not been thoroughly studied.
Prevention of lymphedema
Preventive measures include avoiding exposure to high temperatures, vigorous exercise, and tight clothing (including air cuffs) on the injured limb. Particular attention should be paid to skin and nail care. Vaccinations, phlebotomy, and catheterization of veins in the injured limb should be avoided.
For lymphangitis, antibiotics that are resistant to b-lactamase and effective against gram-positive infectious agents (for example, oxacillin, cloxacillin, dicloxacillin) are prescribed.