Lymphedema is limb edema caused by lymphatic hypoplasia (primary) or obstruction or disturbance of lymphatic channels (secondary). Brawny, fibrous, nonpitting edema in one or more extremities are symptoms and signs. Physical examination is used to make a diagnosis. Exercise, pressure gradient dressings, massage, and, in certain cases, surgery are used to treat the condition. Although a cure is unlikely, treatment can help to alleviate symptoms, delay progression, and prevent consequences. Cellulitis, lymphangitis, and lymphangiosarcoma are all possible side effects.
Etiology of Lymphedema
Primary lymphedemas are rare and hereditary. They differ in terms of phenotype and patient age at the time of presentation.
Congenital lymphedema is caused by lymphatic aplasia or hypoplasia and develops before the age of two. Milroy disease is an autosomal dominant familial form of congenital lymphedema caused by vascular endothelial growth factor receptor-3 (VEGFR-3) gene mutations. It is sometimes associated with cholestatic jaundice and edema, as well as diarrhea caused by intestinal lymphangiectasia.
Lymphedema praecox affects women between the ages of 2 and 35, usually around the start of menstruation or during pregnancy.
Meige disease is an autosomal dominant familial lymphedema praecox caused by mutations in the FOXC2 transcription factor gene. It is characterized by additional eyelashes (distichiasis), cleft palate, and edema of the legs, arms, and occasionally the face.
Lymphedema tarda develops after the age of 35. Both familial and sporadic types exist, with no known genetic foundation for either. Clinical signs and symptoms are comparable to lymphedema praecox, but they may be milder.
Lymphedema is common in a number of other hereditary disorders, including
- Turner syndrome
- Pleural effusions and yellow nails are symptoms of yellow nail syndrome.
- Hennekam syndrome is an uncommon congenital disease characterized by intestinal along with another lymphangiectasia, as well as facial abnormalities and intellectual disability.
Primary lymphedema is significantly less prevalent than secondary lymphedema.
The most common reasons are:
- Surgical procedures (especially lymph node dissection, typically for treatment of breast cancer)
- Radiation therapy is a type of treatment that uses a (especially axillary or inguinal)
- A tumor obstructs the lymphatic system.
- Filariasis of the lymphatic system (in developing countries)
- In patients with persistent venous insufficiency, lymph leaking into interstitial tissues can cause mild lymphedema.
Lymphedema Symptoms and Signs
Aching discomfort and a feeling of heaviness or fullness are common secondary lymphedema symptoms.
Soft-tissue edema is the most common symptom, which is graded in three stages:
Stage 1: The edema is pitting, and the affected area usually recovers by morning.
Stage 2: The edema is nonpitting, and early fibrosis is caused by prolonged soft-tissue inflammation.
Stage 3: Due to soft-tissue fibrosis, the edema is severe and irreversible. The swelling is usually unilateral and worsens in hot weather, before menstruation, and after the leg has been in a dependent position for a long time. It can affect any region of the limb (isolated proximal or distal) or the entire extremity, and periarticular edema can limit the range of motion. When lymphedema is caused by medical or surgical therapy, it can cause substantial disability and emotional suffering.
Hyperkeratosis, hyperpigmentation, verrucae, papillomas, and fungal infections are all common skin alterations.
Rarely, the hyperkeratosis on an affected limb becomes severe, giving it the appearance of elephant skin (elephantiasis). Filariasis causes lymphedema more frequently than other causes of lymphedema.
Lymphangitis can develop when germs pass through the body through skin cracks between the toes as a result of fungal infections or cuts on the hand. Lymphangitis is nearly often streptococcal, resulting in erysipelas; however, staphylococcal lymphangitis can also occur. The infected limb becomes red and heated, with red streaks extending proximally from the entrance location and lymphadenopathy developing. The skin only breaks down on rare occasions.
Long-term lymphedema can develop into lymphangiosarcoma (Stewart-Treves syndrome), which is most prevalent in postmastectomy patients and filariasis patients.
- Clinical diagnosis
- CT or MRI
The presence of distinctive soft-tissue edema across the body, as well as additional information from the history and physical examination, usually indicates primary lymphedema.
Physical examination frequently reveals the presence of secondary lymphedema. If secondary lymphedema is suspected, more testing is recommended unless the diagnosis and causation are clear. Lymphatic blockage can be seen using CT and MRI, while lymphatic hypoplasia or sluggish flow can be detected using radionuclide lymphoscintigraphy.
Once lymphedema sets in, it’s rare to find a cure. Treatment and possibly preventive measures can help to alleviate symptoms, delay or stop disease progression, and avoid consequences.
- Primary lymphedema may require surgical reconstruction.
- Fluid mobilization (eg, by elevation and compression, massage, pressure bandages, intermittent pneumatic compression).
- If the quality of life is considerably affected, surgical soft-tissue reduction (removal of subcutaneous fat and fibrous tissue) and rebuilding may be used to treat primary lymphedema.
The treatment of secondary lymphedema entails addressing the underlying cause. Several fluid mobilization techniques (complex decongestive therapy) can be utilized to treat lymphedema. They include the following:
- The leg is lifted and compressed (“milked”) toward the heart in manual lymphatic drainage.
- Pressure sleeve or bandages with a gradient.
- Exercises for the limbs
- Massage of the limbs with intermittent pneumatic compression.
Surgical soft-tissue reduction, lymphatic anastomoses, and drainage channel construction have all been tried but not thoroughly investigated.
Avoiding heat, intense exercise, and wearing constrictive clothes (such as blood pressure cuffs) around the affected limb are also preventative methods. Vaccination, phlebotomy, and IV catheterization in the affected limb should be avoided; skin and nail maintenance require special attention.
Antibiotics that are beta-lactamase resistant and effective against gram-positive pathogens are used to treat cellulitis and lymphangitis (eg, dicloxacillin).
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