Lymphoedema

From dermnetnz

What is lymphoedema?

Lymphoedema (American spelling ‘lymphedema’) is a swelling of part or parts of the body that occurs when the lymphatic system is not working properly. To understand how lymphoedema occurs we need to know a bit about what the lymphatic system is and how it works.

What is the lymphatic system?

The lymphatic system forms part of the immune system and part of the vascular system. It is made up of many tiny vessels that run throughout the body and gradually join together to form bigger vessels. Running through these vessels is a clear fluid called lymph. Lymph contains the excess protein, water, bacteria and waste products that the body doesn’t require and travels along the lymphatic system until it reaches the lymph nodes (glands). The lymph nodes, which are situated in the neck, armpits, groins and deep inside the chest and abdomen, filter and cleanse the lymph to remove any foreign matter (e.g. bacteria) and start any necessary immune reactions. Once passed through the lymph nodes, lymph finally drains into large veins located in the lower neck. Movement of adjacent muscles helps to keep the lymph moving along the lymphatic system. In addition the walls of the lymphatic vessels contract and valves inside the vessels keep things pumping.

Why does lymphoedema occur?

Oedema (swelling) occurs when excess fluid and protein from damaged blood vessels (e.g. after a burn or ankle sprain) enters into the lymphatic system at a rate too fast for the lymph to be carried away. This produces a temporary swelling which gradually resolves on its own as the damaged tissue heals, blood vessels no longer leak excessively and the lymphatic system can keep pace with the normal flow of lymph.

Lymphoedema is the name given to the swelling that occurs because of a damaged or blocked lymphatic system. Protein, water and waste products are released into tissue in the normal way but a damaged lymphatic system prevents the drainage away and swelling of the affected area results. In addition, the limb may become inflamed. There is an increased risk of infection.

There are basically two types of lymphoedema based on the underlying causes. Primary lymphoedema is also known as Milroy disease.

Primary lymphoedemaSecondary lymphoedemaDamaged lymphatic system is usually present at birth (usually as a result of underdevelopment)Affects both sexes, although 70-80% of cases are femaleAge of onset varies – swelling is present at birth in 10% of cases, 80% develop swelling before 35 years and 10% after 35 yearsCommonest sites for swelling are the legs, arms, genitals and faceImpaired functioning of the lymphatic system is caused by some other condition or factorChronic venous disease – due to incompetent superficial and/or deep veins in the lower legs resulting in venous hypertension (increased pressure) and secondary damage to the lymphatic systemSurgery – removal of lymph nodes when treating some cancers, e.g. breast, prostateRadiotherapy – used to treat cancer but can cause scar tissue that interrupts lymphatic flowAccidental trauma, injury or infection that directly damages lymph vesselsReduced mobility – movement keeps the muscle contracting which helps the flow of lymph

What are the clinical features of lymphoedema?

Initially the symptoms and signs of lymphoedema may be very slight and almost unnoticeable. If left untreated, the swelling may get bigger and eventually become permanent. Some or all of the following symptoms may be experienced.

Feeling of tightness and heaviness in a limbAltered sensation, such as pins and needles, shooting pains or feeling of heatJoint discomfort due to the swelling, e.g. elbow, kneeTenderness in the groin of an affected legChanges in temperature of the limb or affected skinReduced range of movement

At first the swollen area will pit if pressed with a thumb. However, as it becomes bigger and harder it will no longer pit. If lymphoedema is left untreated and the swelling gets worse, skin changes may occur. The skin may get very thick with folds, bulges and dry warty spots (elephantiasis nostras verruciformis, or lymphatic papillomatosis) and make it prone to infection (cellulitis). This is particularly the case if lymph leaks through to the skin.

Lymphoedema of the legs is often worse than that of the arms as lymphatic drainage from the legs is more difficult. Walking becomes difficult as the patient carries the excess weight.

Elephantiasis nostras verruciformis

CellulitisComplications of lymphoedema

How is the diagnosis made?

Diagnosis of lymphoedema is made from a patient's history and observation. The possibility of cancer should be investigated if a cause cannot be found for swelling. In some difficult cases a radiographic test called lymphoscintigraphy may be performed. This measures lymphatic function and gives a fair image of the lymphatic system.

Lymphoedema should be distinguished fromlipoedema, in which swelling of both thighs and lower legs is due to the accumulation of fat under the skin.

What is the treatment for lymphoedema?

Lymphoedema is an ongoing condition that has no known cure. The main goal of treatment is to reduce the swelling and keep it to a minimum. It is very important to treat lymphoedema in its early stages to prevent further swelling and complications such as infection. If a cause is found then the cause needs to be treated, e.g. cancer.

For patients with mild to moderate swelling of their limbs a treatment regimen called Complex Physical (Lymphatic/Lymphoedema) Therapy (C.P.T./C.L.T.) is recommended. This regimen consists of 4 main parts.

Skin care – this involves keeping the skin clean to reduce infection, the use of emollients to restore and maintain skin suppleness, and keratolytics to remove hard, dead skin.Simple lymphatic drainage – this is a gentle massage technique that uses simple hand movements to try to move the swelling out of the affected area. The patient, relative or caregiver is taught the technique.Compression bandaging, sleeve or stocking to help prevent swelling from building up in the limb again.Exercise and movement – specially designed programme to supplement the massage and help by maximising lymph drainage without over exertion

For people with more severe swelling, additional treatments are usually recommended. These include:

Manual Lymphatic Drainage – specialised form of massage carried out by a trained therapist to move the skin in specific directions based on the underlying anatomy and physiology of the lymphatic system.Multi-Layer Lymphoedema Bandaging – multi-layer system of compression bandagesDrug therapy – research is ongoing as to the effectiveness of drug therapy in lymphoedema. Benzopyrenes including flavonoids have been used. Coumarin use has been discontinued due to hepatic toxicity.Surgery – rarely performed but may be of benefit for eyelid or genital swelling. Only surgeons who have experience with lymphoedema and the lymphatic system should perform surgery.

Related information

References:

Book: Textbook of Dermatology. Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. Fourth edition. Blackwell Scientific Publications.Milroy's disease – GeneTests GeneReviewsKerchner K, Fleischer A, Yosipovitch G. Lower extremity lymphedema update: pathophysiology, diagnosis, and treatment guidelines. J Am Acad Dermatol. 2008 Aug;59(2):324-31. Medline.