Patients manifest with a chronic swelling and fullness of the affected limb. Most commonly it is the lower limb and the upper extremity that are affected; trunk and genitalia are less frequently affected. The patient notices asymmetry of the extremity. This progresses to increased heaviness of the limb, fatigue and difficulty in wearing clothes. There is severe deterioration in performing daily activities. The swelling of the limb is usually painless though some patients do report of constant aching in that area.

As the edema becomes chronic and long standing it develops discoloration, the skin becomes dense and appears thick. The texture appears as that of an orange peel. There is fibrous tissue and scar formation.

In addition to this, recurrent fever with chills, weakness, and repeated cellulitis and infections are common. Locally, there might be fissuring, recurrent ulcer formation, and lymphorrhea [6]. Lymphorrhea constitutes of oozing of clear or straw colored fluid from the affected limb. Inability to pinch the affected skin in between two fingers is called as the positive Stemmer test, and is useful in eliciting the diagnosis for lymphedema.

The degree of severity, from mild to severe forms is accessed through a staging method introduced by the Lee, Morgan and Bergan through the American society of lymphology. This method benefits the physician in indentifying the severity of the case and in providing the necessary intervention accordingly.

• Stage 0: Absent lymphedema. This is a latent stage. Here, the lymphatic vessels are destroyed to a mild degree and hence it is not yet apparent.
• Stage 1: The edema is visible but is reversible. It resolves on elevating the limb. Also, the affected area is normal in morning and swelling appears as the day progresses. 
• Stage 2: The tissues are affected. They become non-pitting on pressure. Lymphedema is evident with increasing swelling and hardening of the skin. 
• Stage 3: Irreversible swelling is seen in this stage. The limb tissue becomes hard and non tender. There is marked heaviness and disability of the limb. There is complete blockage of the lymphatic vessels. This is also called as stage of elephantiasis [4] [7].

• Lymphedema

  […] primary lymphedema lymphedema that appears spontaneously without known cause. [medical-dictionary.thefreedictionary.com]

  Our lymphedema therapists can help you understand: Causes of lymphedema, treatment options and who is at risk How diet and exercise affect lymphedema How to use to massage to decrease lymphedema [franciscanhealth.org]

  Some patients are born with swelling in the extremities due to lymphedema and others develop lymphedema later in life. [tennesseeveincare.com]

  Primary lymphedema is caused by developmental lymphatic vascular anomalies. Secondary lymphedema is acquired and arises as a result of an underlying systemic disease, trauma, or surgery. [ncbi.nlm.nih.gov]

• Swelling

  Symptoms of lymphedema include swelling in one or more extremities. The swelling may range from mild to severe and disfiguring. [medicinenet.com]

  This accumulation of fluid results in abnormal swelling of the arm(s) or leg(s), and occasionally swelling in other parts of the body. Lymphedema is a very serious condition. [medical-dictionary.thefreedictionary.com]

  Lymphedema is a persistent swelling of a body part due to a blockage in lymphatic flow when nodes or vessels are damaged or abnormal. The swelling may result in pain, decreased range of motion, and/or weakness. [beyondbasicsphysicaltherapy.com]

  Specialized Relief from the Swelling and Discomfort of Lymphedema Lymphedema is a progressive disease that causes swelling in your legs, arms, groin or stomach. Without treatment, the swelling, known as lymphedema, may continue to increase. [franciscanhealth.org]

  Some patients are born with swelling in the extremities due to lymphedema and others develop lymphedema later in life. [tennesseeveincare.com]

• Pain

  This results in pain and also known as painful fat syndrome. It involves the hips, thighs, and ankles; the feet are not affected. Lipedema appears as a symmetrical ring of tissue around the extremity, and doesn't respond to weight loss. [beyondbasicsphysicaltherapy.com]

  Learn more about pain management [cancercenter.com]

  Pain Management Approaches for Lymphedema Neurostimulation involves stimulating the nervous system or giving medications that directly affect the nervous system to relieve pain. [mskcc.org]

  In addition to the swelling, other symptoms can include: Warmth, redness, or itching Tingling or burning pains Fever and chills Decreased flexibility in the joints Aching, pain, and fullness in the affected area Skin rash Suppressed immune function in [medicinenet.com]

  Pain bursts in the affected limb. Pain in the buttocks (leg lymphedema) or behind the shoulder (arm lymphedema). Tingling in affected members. Shooting pain through the affected limb. [maximedtherapy.com]

• Inflammation

  Any infection or inflammation of intervention or even a sunburn may worsen lymphedema. [maximedtherapy.com]

  Lymphedema occurs when the lymphatic system is not functioning properly due to a defect in the lymph vessels present at birth or from damage to the lymph system from surgery, chronic venous insufficiency, radiation, trauma or chronic inflammation or infection [franciscanhealth.org]

  Inflammation of the skin and connective tissues, known as cellulitis, and inflammation of the lymphatic vessels (lymphangitis) are common complications of lymphedema. [medicinenet.com]

  Other symptoms include inflammation, disfigurement, decreased mobility and limb function, skin breakdown or infection, and fat deposits. [aapmr.org]

  Histopathologic examination revealed pleomorphic perivascular and perilymphatic inflammation with profound lymphangiectasis and lymph stasis, thus suggesting elements of both rosacea and localized, chronic lymphedema. [ncbi.nlm.nih.gov]

• Surgical Procedure

  Any type of surgical procedure that requires removal of regional lymph nodes or lymph vessels can potentially cause lymphedema. [medicinenet.com]

  When applied appropriately to properly selected patients, surgical procedures used in the treatment of lymphedema are effective and safe. [ncbi.nlm.nih.gov]

  Abnormalities of the lymphatic channels present at birth result in congenital lymphedema; others will develop lymphedema due to obstruction from recurring cellulitis or soft tissue infections, surgical procedures, or compression by abdominal obesity or [tennesseeveincare.com]

  → Read More: On the Importance of Lymphedema Screening for Upper and Lower Extremities Following Surgical Procedures By Joachim Zuther, on October 26th, 2018 Tweet Successful lymphedema management requires a well-trained lymphedema therapist and a properly [lymphedemablog.com]

• Nausea

  DON’T be afraid to ask for medicine for pain, nausea, or vomiting. [spectrumhealth.org]

  nausea in some patients. [doi.org]

  Gastrointestinal Tract Symptoms of gastrointestinal lymphoma may include: Abdominal tenderness and pain Cramps Indigestion Constipation Diarrhea Malaise (a general feeling of unwellness) Early satiation (a feeling of fullness after a few bites) Nausea [verywellhealth.com]

  This can be a tough process, because you may get side effects like nausea and mouth sores. Certain medications can make these side effects less severe. When the high-dose chemo is done, you'll start the transplant. [webmd.com]

  Acupuncture has been helpful in relieving musculoskeletal symptoms, as well as in controlling nausea and vomiting associated with chemotherapy. [emedicinehealth.com]

• Vomiting

  DON’T be afraid to ask for medicine for pain, nausea, or vomiting. [spectrumhealth.org]

  Other common lymphoma symptoms include: Fever Fatigue or lethargy Loss of appetite or vomiting Sudden and dramatic weight loss Night sweats or chills Unexplained pain or swelling Difficulty moving body parts Lymphoma stages At CTCA®, a thorough and accurate [cancercenter.com]

  Symptoms of liver lymphoma are often mild and non-specific and may include: Extreme fatigue Pain or swelling in the upper right abdomen Loss of appetite Nausea and vomiting Jaundice (yellowing of the skin and/or eyes) Dark urine Unintended weight loss [verywellhealth.com]

  If emesis is severe it can in turn lead to anticipatory nausea and vomiting ( Morrow 1998 ). [doi.org]

  Vomiting, fever, or chills may accompany this infection. If there is any question of cellulitis, a health care provider should be consulted promptly so that appropriate antibiotic therapy can be initiated. Figure 2. [journals.sagepub.com]

• Constipation

  […] of fullness in the limb Tight-feeling skin Decreased flexibility in the affected joint Difficulty fitting into clothing in one specific area Ring, bracelet, wristwatch, shoe or sock tightness Fatigue Breast pain Fibromyalgia Infertility Painful periods Constipation [beyondbasicsphysicaltherapy.com]

  Gastrointestinal Tract Symptoms of gastrointestinal lymphoma may include: Abdominal tenderness and pain Cramps Indigestion Constipation Diarrhea Malaise (a general feeling of unwellness) Early satiation (a feeling of fullness after a few bites) Nausea [verywellhealth.com]

• Heart Disease

  Disease Cuts Both Ways AHA News: Woman's Frozen Face, Garbled Speech During Zoom Call Were Signs of a Stroke View All News > View All News > ABOUT Overview History Editors and the Editorial Process Publishing and Production Staff Editorial Board Contributors [msdmanuals.com]

  Heart Disease Heart disease is believed to be the leading, non-cancer cause of death in people with lymphoma. [verywellhealth.com]

  The type of exercise that is best for an individual depends upon the severity and cause of lymphedema and other co-existing medical conditions (e.g. heart disease, diabetes, arthritis, etc). [web.archive.org]

  Your doctor may perform other tests to rule out heart disease, blood clots, infection, liver or kidney failure, or an allergic reaction. Stages of lymphedema Doctors describe lymphedema according to its stage, from mild to severe: Stage 0. [cancer.net]

• Chest Pain

  Non-Hodgkin lymphoma can cause many symptoms, such as Swollen, painless lymph nodes in the neck, armpits or groin Unexplained weight loss Fever Soaking night sweats Coughing, trouble breathing or chest pain Weakness and tiredness that don't go away Pain [medlineplus.gov]

  DO call your health care provider if you have pain, fever, or drainage from the incision after surgery. DO call your health care provider if you have shortness of breath, chest pain, or coughing with a fever after radiation. [spectrumhealth.org]

  Symptoms of pulmonary lymphoma are often non-specific in the early stages of the disease and may include: Coughing Chest pain Fever Shortness of breath Crepitus (audible lung crackles) Hemoptysis (coughing up blood) Unintended weight loss With advanced [verywellhealth.com]

  One patient had a small pulmonary embolus on the first postoperative day that caused localized chest pain but no physiologic sequelae. [doi.org]

• Arm Swelling

  OBJECTIVES: The aim of the study was to evaluate the potential role of light arm compression sleeves for reducing the incidence of early postoperative swelling and of breast cancer-related arm LE. [ncbi.nlm.nih.gov]

  Overweight, oblique surgical incision, infection in the arm, and radiotherapy correlated with arm swelling. [doi.org]

  If you have arm swelling, wear long oven mitts when using the oven or stove For upper limb edema, wear gloves when gardening, and long pants or use knees pads if you have lower limb edema Avoid overuse of the arm by using two arms for carrying or carry [uwhealth.org]

  Lymphedema is the chronic swelling or feeling of tightness in the arm or hand due to an accumulation of lymphatic fluid in the soft tissue of the arm. [imaginis.com]

• Edema of the Upper Extremity

  increase in upper-extremity girth, and heaviness. [cancer.gov]

• Muscle Strain

  Muscle strain. Binding forces. A genetic defect in the lymphatic system. It is unclear why some patients develop lymphedema, and others do not. What are the symptoms of lymphedema? Symptoms of lymphedema include: Swelling. Feeling of heaviness. [womenandinfants.org]

• Muscle Spasm

  English 1) The following adverse reactions have a higher frequency category as compared to MEK115306: muscle spasm (very common); renal failure and lymphoedema (common); acute renal failure (uncommon); 2) The following adverse reactions have occurred [mymemory.translated.net]

• Hyperkeratosis

  Stage III lymphedema is characterized by a tremendous increase in volume, hardening of the dermal tissues, hyperkeratosis, and papillomas of the skin. [nortonschool.com]

  Skin changes are common and include hyperkeratosis, hyperpigmentation, verrucae, papillomas, and fungal infections. [msdmanuals.com]

  […] insufficiency Lymphedema of lip Lymphedema of lower extremity Lymphedema of penis Lymphedema of scrotum Lymphedema of uncertain etiology Lymphedema of vulva Lymphedema praecox Lymphedema, after surgery Lymphedema, limb Lymphedema, lower extremity Lymphedematous hyperkeratosis [icd9data.com]

  Dryness, increased thickness, hyperkeratosis, lymphangiomas (blisters containing lymph fluid), abnormally warm or hot, unusually dark skin, and any nodules are all indicators of lymphedema. [ncbi.nlm.nih.gov]

• Eczema

  […] nodes are removed Radiation therapy that can damage nearby tissue that might include lymph nodes or lymph vessels Infections that damage surrounding tissue or cause scarring Other health conditions, such as heart or vascular disease, arthritis, and eczema [cancer.org]

  Conditions that can cause lymphoedema include: rheumatoid arthritis eczema Venous diseases Diseases that affect the flow of blood through the veins can cause lymphoedema in some people. [nhs.uk]

  On DermNet NZ: Leg ulcers Gravitational eczema Lipoedema Morbihan disease Vulval cancer Other websites: The Lymphoedema Association of Australia British Lymphology Society Lymphoedema Support Network (UK) Australasian Lymphology Association Milroy disease [dermnetnz.org]

  Inflammatory conditions: Conditions that cause tissue to swell (become inflamed) may permanently damage the lymphatic system, such as rheumatoid arthritis and eczema. Cardiovascular diseases: These are diseases that affect blood flow. [medicalnewstoday.com]

  […] following: Varicose veins Itching (pruritus) Hyperpigmentation Phlebetic lymphedema[5] Chronic swelling of the legs and ankles Venous ulceration CVI in the leg may cause the following: Venous stasis Ulcers.[6] Stasis dermatitis,[6] also known as varicose eczema [en.wikipedia.org]

• Pruritus

  Signs and symptoms[edit] Chronic venous insufficiency Signs and symptoms of CVI in the leg include the following: Varicose veins Itching (pruritus) Hyperpigmentation Phlebetic lymphedema[5] Chronic swelling of the legs and ankles Venous ulceration CVI [en.wikipedia.org]

  In addition, skin dryness and pruritus also need to be addressed. All patients should be seen by a wound care nurse if there is tissue breakdown. At this point, the chances of healing are small, and daily wound dressings are necessary. [ncbi.nlm.nih.gov]

  Symptoms of lymphoma may vary from patient to patient and may include one or more the following: Fevers Chills Unexplained weight loss Night sweats Lack of energy Itching (up to 25% of patients develop this itch [pruritus], most commonly in the lower [emedicinehealth.com]

• Headache

  Migraine headache, chronic venous insufficiencies and edema of other genesis present additional indications. MLD also has a detoxifying effect. [massagetoday.com]

  Common signs and symptoms of lymphoma Lump in your armpit or neck Night sweats Fever or chills Unexplained weight loss Loss of appetite Shortness of breath Itching (25% of patients) Headache or pain in the back or bones Most of these symptoms can be mistaken [womenshealthnetwork.com]

  It can cause a variety of symptoms, including headaches, changes in vision, weight gain, and edema. Preeclampsia that occurs during pregnancy can also affect the fetus. It requires urgent medical treatment. [medicalnewstoday.com]

  Symptoms of primary or secondary CNS lymphoma include: Headaches Muscle weakness in a specific body part Loss of sensation in a specific body part Problems with balance, memory, cognition, and/or language Changes in vision or partial vision loss Nausea [verywellhealth.com]

• Renal Insufficiency

  The typical appearance of various edema syndromes (primary lymphedema, secondary lipolymphedema, venous insufficiency, and edema due to heart or renal failure – see Table 1) – are illustrated in Figures 3, 4, 5, 7, and 11. [o-wm.com]

Blood tests include complete blood count, liver function tests, urine examination, creatinine and urea levels including electrolytes. These tests help in identifying the underlying infectious etiology.

Imaging studies such as lymphangiography might be done; it is an invasive technique that evaluates the lymphatic channels and their patency. This technique causes a lot of adverse reactions such as atrophy, fibrosis and obliteration; thus it is not advised frequently. Ultrasonography identifies structural and capacitative changes within the lymphatic vessels. A CT scan and MRI are done in cases of a suspected malignancy [8] [9].

• Staphylococcus Aureus

  Clinically mild encephalitis/encephalopathy with a reversible splenial lesion caused by methicillin-sensitive Staphylococcus aureus bacteremia with toxic shock syndrome: a case report. BMC Infect Dis 2016;16:160. 6. Takanashi J. [e-jnc.org]

Treatment comprises of use of compression bandages and garments including stockings. These are applied for several days so that tension is maintained in order to reduce lymphedema. These bandages should be costume fit and should not produce a tourniquet effect. These should be worn during the day and during activities short bandages are preferred that improve the pumping activities against increasing resistance [5] [9].

CDT is complete decongestive therapy and consists of a combination of exercises, bandaging and after skin care. Here, under expert guidance, manual massaging of the limb is done to improve circulation and lymph flow. The treatment is highly effective and enhances drainage.

Intermittent pump therapy involves use of multi-chambered pump compression that causes effective drainage of the lymph.

Surgery offers palliation. It helps to reduce the load of the swelling and is useful for patients who did not respond to the conservative treatment method. Appropriate hygiene is very important to observe in cases of lymphedema. Repeatedly cleansing of the area is recommended in order to eliminate dead skin and debris and prevent the invasion of bacterial or other microbial infections. Patients are advised to lose weight, monitor the limb regularly and to wear proper clothing and comfortable foot wear [10].

Prognosis depends on the severity of the lymphedema, the general health of the patient and the underlying conditions that have caused it.

Long standing lymphedema may develop into a lymphangiosarcoma. This tumor has a very poor prognosis and requires amputation at its earliest. Kaposi sarcoma, B-cell lymphoma are other tumors found with chronic lymphedema.

Lymphedema may also lead to complications such as cellulitis, bacterial infections or deep vein thrombosis leading to functional impairment and disability [5].

Based on the causes, lymphedema can be categorized into primary and secondary lymphedema.

Primary causes are related to congenital or developmental arrest of the lymphatic system. In congenital lymphedema, there is failure of the lymphatic channels to develop. This is an autosomal-dominant disorder. The genetic defect mutated and is mapped into the telomeric part of chromosome 5. This most commonly affects bilateral lower extremities. Meige disease or lymphedema praecox is a common form of primary lymphedema. This is more frequently found after a birth defect. Lymphedema tarda is due to valvular incompetency. This is found in individuals older than 35 years [2].

Secondary lymphedema is obtained due to underlying conditions such as infection, trauma, obesity or a tumor mass. This condition obstructs the lymph flow or damages the vessels carrying the lymphatic fluid, leading to an abnormal collection, producing a swelling and edema of the affected arm or leg.

Studies reveal that lymphedema usually follows after a breast cancer surgery. Around 80% of the cases show congenital onset. Demographics reveal that lymphedema that is primary in nature is seen from birth up to 1 year of age and the secondary ones can occur at any age.

Amongst the infections, filariasis is the most frequent cause leading to lymphedema. Obesity has been highly associated with lymphedema.

Lymphatics normally drain proteins, lipids and water from the interstial tissues into the circulation filtering via the lymph nodes. This occurs through osmotic mechanism. The flow of the lymph is controlled by the valves within the vessels to prevent the back flow of the fluid, also the surrounding muscle contraction and the pulse pressure are important factors involved with the lymph flow.

In case of lymphedema, the transport volume is reduced. The amount of fluid formation exceeds to the volume of fluid returned [3]. Consequently there is stagnation, edema formation with high concentration of proteins per ml. Eventually, the lymphatic vessels undergo changes in the form of fibrosis, thrombus formation and shunt formation.

Within the interstitial spaces, fluid accumulation leads to inflammation producing increased macrophage activity and collagen deposition. This eventually progresses to a suppressed immune activity that increases probabilities of infection and degeneration.

Locally, the skin appears congested and thick; the characteristic appearance is that of an orange skin called peau d’orange appearance. The epidermal layer of the skin gets destroyed and develops fissures, resulting in leakage of the lymph on the surface of the skin. Bacteria enter and multiply leading to a condition called elephantiasis verrucosa [4].

Those at a risk for developing lymphedema should observe special precautions especially after undergoing a cancer surgery. Injury to the limb from sharp objects should be avoided. Regular exercise and elevation of the affected limb during rest is advisable. Tight garments hamper circulation and cause constriction; hence comfortable size garments should be worn. Lastly, skin care and hygiene must be observed regularly to avoid infection [1] [11].

The lymphatic fluid and vessels essay a very vital role in the body’s immune system. The lymphatic circulation consists of many lymph nodes and lymphatic vessels through which the lymphatic fluids pass [1]. It acts by draining the unwanted lymphatic fluid from tissues of the body in the blood circulation for removal.

When this lymphatic fluid is abnormally accumulated within the interstitium, it results in swelling under the skin leading to edema called as lymphedema.

Lymphedema develops in cases where normal lymph drainage is obstructed due to congenital causes or underlying tumors, after surgery or trauma, the fluid builds up causing increased swelling and discomfort.

Long standing lymphedema increases the risk of infection, cellulitis and fibrosis. Treatment includes use of compression bandages and garments like wearing stockings and bandages. These help in improving lymph drainage and circulation. Secondly, gentle exercises, elevation of the limb while resting are advised to enhance lymph drainage. Surgery is advised in cases when conservative treatment does not respond. Other precautions observed are protection of the limb from any injury especially from sharp objects and needles.

1. Foldi E, et al. The Science of Lymphoedema Bandaging in Calne, S. Editor. European Wound Management 2005Association (EWMA). Focus Document: Lymphoedema Bandaging in Practice. London: MEP Ltd, 2004.
2. Casley-Smith JR. Alterations of untreated lymphedema and its grades over time. Lymphology. 1995 Dec; 28(4):174-185
3. MacLaren JA. Skin changes in lymphoedema: pathophysiology and management options. Int J Palliat Nurs. 2001 Aug;7(8): 381-388
4. Pereira de Godoy JM, Azoubel LM, de Fatima Guerreiro de Godoy M. Intensive treatment of leg lymphedema. Indian J Dermatol. 2010 Apr-Jun;55(2):144-7
5. Elgendy IY, Lo MC. Unilateral lower extremity swelling as a rare presentation of non-Hodgkin's lymphoma.BMJ Case Rep. 2014 Jan 15; 2014. pii: bcr2013202424.
6. Fife C. Massive localized lymphedema, a disease unique to the morbidly obese: a case study. Ostomy Wound Manage. 2014 Jan;60(1):30-5.
7. Prakash J, Kumar M, Singh V, Sankhwar S. Giant penile elephantiasis after circumcision: a devastating complication. BMJ Case Rep. 2013 Sep 16; 2013 pii:bcr2013200780.
8. Namnyak S, Adhami Z, Toms G, Jenks P. Pasteurella multocida septicaemia in Milroy's disease. J Infect. 1995 Sep;31(2):175-6.
9. Zuther JE. Lymphedema Management: The Comprehensive Guide for Practitioners. 2nd ed. New York, NY: Thieme; 2009.
10. Connell F, Brice G, Jeffery S, Keeley V, Mortimer P, Mansour S. A new classification system for primary lymphatic dysplasias based on phenotype. Clin Genet. 2010 May;77(5):438-52.
11. Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci. 2008;1131:140-6.