Lymphedema: causes, symptoms, diagnosis, treatment

Lymphedema - edema of the 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 (when you press a finger does not leave an impression) swelling of one or more extremities. Diagnosis is established during a physical examination. Treatment of lymphedema consists of physical exercises, wearing compression tights / stockings, massage and (sometimes) surgical intervention. A complete cure usually does not occur, but treatment can reduce symptoms and slow or stop the progression of the disease. Patients have a risk of developing panniculitis, lymphangitis, and (rarely) lymphangiosarcoma.

Lymphedema may be primary (arising from hypoplasia of the lymphatic vessel) or secondary (developing as a result of obstruction or destruction of the lymphatic vessels).

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Primary lymphedema

Primary lymphedema is hereditary in nature and is rare. They are different in phenotypic manifestations and in age, in which the first manifestations appear.

Congenital lymphedema appears before the age of 2 years and arises from hypoplasia or aplasia of the lymphatic vessels. Milroy's disease is an autosomal dominant hereditary form of congenital lymphedema attributed to mutations of VEGF3 and sometimes associated with cholestatic jaundice and edema or diarrhea due to enteropathy with protein loss caused by lymphatic stasis in the intestinal tissues.

The main amount of lymphedema appears in the age range of 2 to 35 years, women typically debut in establishing menstruation or the occurrence of pregnancy. Maize disease is an autosomal dominant hereditary form of early lymphedema attributed to mutations of the gene transcription factor (F0XC2) and associated with the presence of a second series of eyelashes (dysthiasis), wolf mouth and swelling of the foot, hands and sometimes face.

The debut of late lymphedema occurs after 35 years. There are family and sporadic forms, genetic aspects are unknown. Clinical findings are similar to those with early lymphedema, but may be less pronounced.

Lymphedema occurs in a variety of genetic syndromes, including Turner syndrome, yellow nail syndrome (characterized by pleural effusions and yellow nails), and Hennecam's syndrome, a rare congenital syndrome characterized by the presence of intestinal (or other localization), facial skull anomalies and oligophrenia.

Secondary lymphedema

Secondary lymphedema is a much more common pathology than the primary one. Its causes are usually surgical intervention (especially the removal of lymph nodes typical for breast cancer operations), radiation therapy (especially underarm or inguinal area), trauma, lymphatic vessel obstruction by the tumor and (in developing countries) lymphatic filariasis. Moderate lymphedema can also develop with lymphatic flow to the interstitial structures in patients with chronic venous insufficiency.

Symptoms of secondary lymphedema include painful discomfort and a feeling of heaviness or overflow in the affected limb.

The cardinal symptom is swelling of the soft tissues, subdivided by the sodium stage.

• In stage I, after pressing in the edema zone, pits remain, and the damaged area returns to normal by morning.
• In the II stage of the pits after pressing with a finger does not remain, and chronic inflammation of the soft tissues causes early fibrosis.
• In the third stage, the skin over the affected area becomes brown and the edema is irreversible, largely due to fibrosis of the soft tissues.

Swelling is usually one-sided and may increase in warm weather, before menstrual bleeding and after a long stay of the limb in a forced position. The process can affect any part of the limb (isolated proximal or distal lymphedema) or the entire limb. Limits of range of movements are possible if the edema is localized around the joint. Disturbance of functions and emotional stress can be significant, especially if lymphedema has arisen as a result of medical or surgical treatment.

Frequent skin changes include hyperkeratosis, hyperpigmentation, warts, papillomas and fungal infections.

Lymphangitis usually develops when bacteria enter through the skin cracks between the toes as a result of mycosis or through cuts on the hands. Lymphangitis is almost always streptococcal and causes a face, sometimes they detect staphylococci.

The damaged limb becomes hyperemic and hot, the red bands can extend proximally to the focus. Lymphadenopathy is possible. Sometimes cracks appear on the skin.

Diagnosis of lymphedema

The diagnosis is usually obvious in a physical examination. Additional studies are prescribed for suspected secondary lymphedema. With CT and MRI, it is possible to identify sites of lymphatic vessel obstruction. Radionuclide lymphoscintigraphy helps detect lymphatic hypoplasia or a decreased rate of lymph flow. Negative dynamics can be detected by measuring the circumference of a limb, determining the volume of fluid displaced when the limb is immersed in water, or using dermal or tonometry soft tissue; these tests were not approved. In developing countries, research is needed on lymphatic filariasis.

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Prognosis and treatment of lymphedema

Complete cure is not characteristic if lymphedema develops. Optimum treatment and (possibly) preventive measures can somewhat level the symptoms and slow or stop the progression of the disease. Sometimes prolonged lymphedema leads to lymphangiosarcoma (Stewart-Trivs syndrome), usually in women after mastectomy and in patients with filariasis.

Treatment of primary lymphedema can include excision of soft tissues and reconstruction of lymphatic vessels, if this leads to a significant improvement in the quality of life.

Treatment of secondary lymphedema involves the possible elimination of its cause. To eliminate the symptoms of lymphedema, several techniques can be used to remove excess fluid from the tissues (complex anti-edema therapy). These include manual lymphatic drainage, in which the limb rises, and blood pressure is directed toward the heart; the pressure gradient is maintained by limb dressing, performing exercises, limb massage, including periodic pneumatic compression. Sometimes surgical resection of soft tissues, the creation of additional anastomoses of lymphatic vessels and the formation of drainage canals, but the methods have not been thoroughly studied.

Prevention of lymphedema

Preventive measures include the exclusion of high-temperature exposure, vigorous exercise and squeezing clothing items (including pneumatic cuffs) worn on the injured limb. Particular attention requires care for the condition of the skin and nails. Vaccination, venotomy and catheterization of the veins of the injured limb should be avoided.

With lymphangitis, antibiotics are prescribed that are resistant to b-lactamase and are effective against gram-positive infectious agents (eg, oxacillin, cloxacillin, dicloxacillin).