According the Fifth WHO Expert Committee on Filariasis , the most common method of classification of lymphedema is as follows: (The same classification method can be used for both primary and secondary lymphedema)

The International Society of Lymphology (ISL) Staging System is based solely on subjective symptoms, making it prone to substantial observer bias. Imaging modalities have been suggested as useful adjuncts to the ISL staging to clarify the diagnosis. The lymphedema expert Dr. Ming-Huei Cheng developed a Cheng’s Lymphedema Grading tool to assess the severity of extremity lymphedema based on objective limb measurements and providing appropriate options for management.

Accurate diagnosis and staging are fundamental to the management of lymphedema patients. A swollen limb can result from different conditions that require different treatments. Diagnosis of lymphedema is currently based on history, physical exam, limb measurements, and imaging studies such as lymphoscintigraphy and indocyanine green lymphography. However, the ideal method for lymphedema staging to guide the most appropriate treatment is controversial because of several different proposed protocols.

Lymphedema can occur in both the upper and lower extremities, and in some cases, the head and neck. Assessment of the extremities first begins with a visual inspection. Color, presence of hair, visible veins, size and any sores or ulcerations are noted. Lack of hair may indicate an arterial circulation problem. Given swelling, the extremities' circumference is measured for reference as time continues. In early stages of lymphedema, elevating the limb may reduce or eliminate the swelling. Palpation of the wrist or ankle can determine the degree of swelling; assessment includes a check of the pulses. The axillary or inguinal nodes may be enlarged due to the swelling. Enlargement of the nodes lasting more than three weeks may indicate infection or other illnesses such as sequela from breast cancer surgery requiring further medical attention.

Diagnosis or early detection of lymphedema is difficult. The first signs may be subjective observations such as "my arm feels heavy" or "I have difficulty these days getting rings on and off my fingers". These may be symptomatic of early stage of lymphedema where accumulation of lymph is mild and not detectable by changes in volume or circumference. As lymphedema develops further, definitive diagnosis is commonly based upon an objective measurement of differences between the affected or at-risk limb at the opposite unaffected limb, e.g. in volume or circumference. No generally accepted criterion is definitively diagnostic, although a volume difference of 200 ml between limbs or a 4-cm difference (at a single measurement site or set intervals along the limb) is often used. Bioimpedance measurement (which measures the amount of fluid in a limb) offers greater sensitivity than existing methods.

Chronic venous stasis changes can mimic early lymphedema, but the changes in venous stasis are more often bilateral and symmetric. Lipedema can also mimic lymphedema, however lipedema characteristically spares the feet beginning abruptly at the medial malleoli (ankle level). Lipedema is common in overweight women. As a part of the initial work-up before diagnosing lymphedema, it may be necessary to exclude other potential causes of lower extremity swelling such as renal failure, hypoalbuminemia, congestive heart-failure, protein-losing nephropathy, pulmonary hypertension, obesity, pregnancy and drug-induced edema.

This disease is caused by problems in the circulatory system, so when it is presented, in the beginning it is important to follow several recommendations. The person needs to keep the legs elevated as much as possible to help the return of the blood. Whenever sitting down, the person needs to keep the legs on a foot stool. At night it is advisable to sleep with a pillow under the lower legs. In the evening, t is not unusual for legs to be swollen. The volume of the lower leg can increase to up to 100ml after a long working day or up to 200ml after a long-haul flight without moving.

In the example of the 41-year-old Japanese man the lesions were much improved by washing and topical use of corticosteroids for two months, also oral antibiotics like cephalexin are used if cellulitis is present. Moist exudative inflammation and moist ulcers respond to tepid wet compresses of Burow’s solution or just saline or water for 30 to 60 minutes several times a day. But in worse cases, edema that does not disappear spontaneously within a few hours or after a walk, is described as pathological, so it needs to have a special treatment. It is very important to say that Papillamitosis, bilateral and marked edema with few symptoms is mostly caused by the systemic circulation (heart, kidneys, liver).

Papillamitosis is associated, as has been mentioned before, with symptoms and/or clinical signs such as dilated superficial veins, varicose veins and changes in the skin. Edema and its complication Papillamitosis are only partially reversible and soon becomes hard, which is mainly confirmed on palpation. All skin structures are affected and this is characterized by the term. Lymphoedema may develop in many cases accompanied by acral thickening of the skin folds, hyperkeratosis and papillomatosis.

Diagnosis is through biopsy. The presence of hypoproteinemia, decreased blood lymphocytes, and decreased cholesterol support the diagnosis. Hypocalcemia (low calcium) is also seen due to poor absorption of vitamin D and calcium, and secondary to low protein binding of calcium. Medical ultrasonography may show s in the intestinal mucosa indicating dilated lacteals.

Lipedema is commonly misdiagnosed. At this time, only Germany and the Netherlands have standardized ways of diagnosing lipedema. Other countries do not currently have a standardized diagnosis protocol, and therefore the diagnosis is typically made clinically via physical inspection (palpating the adipose tissue).

Some trained clinicians and therapists can feel the physical differences in the adipose tissue, characterized as nodules with "beans in a bag" feeling. When the tissue has excess fluid the nodules are no longer easily palpable. Testing of the major components of the lymphatic system can be done through tools such as lymphoscintigraphy, but lack of noticeable lymphatic impairment does not indicate lipedema is not present, only that the major lymphatics are not (yet) affected.

Lymphedema is usually asymmetrical and can be either acquired (through surgery, trauma or infection damaging the lymphatic system) or congenital (hereditary changes in the lymphatic system). However, symmetrical enlargement of both lower limbs, from waist to ankles (i.e. gynoid fat) is a hallmark of lipedema. As the swelling continues and spreads from lower extremities to other parts of the body, the swelling is likely caused by slower lymphatic flow and changes in the lymphatic vessel structure caused by pressure in the lipedematous limbs. This is known as lipo-lymphedema. Lipo-lymphedema may also develop in combination with chronic venous insufficiency and other vascular disorders.

Lipedema can be underdiagnosed due the difficulty in differentiating it from other edemas and obesity. Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.

Lipedema / Dercum’s Disease Differentiation

These conditions may co-exist. Dercum’s Disease is characterized by painful lipomas around the body.

Stasis Papillomatosis is similar to AGEP (Acute generalized exanthematous pustulosis) from pustular psoriasis; criteria for histopathologic distinction have been proposed: papillary edema, vasculitis, exocytosis of eosinophils and single-cell necrosis of keratinocytes in AGEP and acanthosis and papillomatosis in pustular psoriasis.

An example that illustrates the difference between SP and Stasis Papillomatosis and the histology diagnosis is … “a markedly obese, 41-year-old Japanese man who had suffered from psoriasis vulgaris for several years visited hospital with elephantiasis-like swelling of his lower legs of three months' duration. His right lower leg showed marked papillomatosis with thick scales, and the left lower leg was eroded and papillomatous. Although direct lymphography of his lower extremities showed no abnormality, indirect lymphography revealed local lymphatic damage in the involved skin”. Histological examination showed hyperkeratosis, marked papillomatosis, proliferation of capillaries in the upper dermis, and lymphectasia in the lower dermis. It was suspected that obesity and the preceding psoriatic lesions caused local lymphatic disturbances, followed by the development of stasis papillomatosis.

Treatment is multifactorial. A diet very low in fat and high in high quality protein is essential. Treatment of humans can also involve the use of MCT (medium-chain triglycerides) oil and/or the drug octreotide. In dogs, fat soluble vitamins (A, D, E, and K) should be supplemented. Corticosteroid treatment may be required for life. Antibiotics can be used to treat bacterial overgrowth. With a very low serum albumin, transfusion with blood plasma or an infusion of hetastarch may be necessary to treat the signs until the diet can take effect. Lymphangiectasia is rarely cured but can remain in remission for a long time. It can be fatal when unresponsive to treatment.

The differential diagnosis for podoconiosis includes other causes of tropical lymphedema, such as filariasis or leprosy, and mycetoma pedis. Podoconiosis begins almost exclusively in the foot, as opposed to filariasis, where the initial edema can appear anywhere in the lower extremities. Podoconiosis is usually asymmetrically bilateral, whereas filariasis and mycetoma are usually unilateral. Additionally, groin involvement with podoconiosis is extremely rare and is usually indicative of filariasis.

If a clinical distinction between podoconiosis and filariasis cannot be made based on history and examination alone, blood smears and ELISA antigen testing can be useful to screen for filariasis.

The disfigurement associated with podoconiosis can include soft or firm edema, and in later stages firm nodules and a mossy appearance, whereas mycetoma is characterized by firm nodules and edema, usually without the mossy appearance of podoconiosis. Additionally, the edema of podoconiosis is typically more striking and extends more proximally than the edema of mycetoma. Radiology can help distinguish between podoconiosis and mycetoma if the diagnosis is questionable.

Local epidemiology can also be a clue to diagnosis, as podoconiosis is typically found in higher altitude areas with volcanic soils, whereas mycetoma is found along the "mycetoma belt" between latitudes 15 south and 30 north, and filariasis is uncommon at higher altitudes and other environments in which the mosquito vector is less prevalent.

Podoconiosis can be distinguished from leprosy by the preservation of sensation in the affected limb and the isolation of disease to the lower extremities.

The risk of the development of a lymphocele is positively correlated to the extent of the removal of lymphatic tissue during surgery (lymphadenectomy). Surgery destroys and disrupts the normal channels of lymph flow. If the injury is minor, collateral channels will transport lymph fluid, but with extensive damage, fluid may accumulate in an anatomic space resulting in a lymphocele. Typical operations leading to lymphocysts are renal transplantation and radical pelvic surgery with lymph node removal because of prostatic or gynecologic cancer. Other factors that may predispose of lymphocele development are preoperative radiation therapy, heparin prophylaxis (used to prevent deep vein thrombosis), and tumor characteristics. After radical surgery for cervical and ovarian cancer studies with follow-up CT found lymphoceles in 20% and 32%, respectively. Typically they develop within 4 months after surgery.

Because it is rare and has a wide spectrum of clinical, histological, and imaging features, diagnosing lymphangiomatosis can be challenging. Plain x-rays reveal the presence of lytic lesions in bones, pathological fractures, interstitial infiltrates in the lungs, and chylous effusions that may be present even when there are no outward symptoms.

The most common locations of lymphangiomatosis are the lungs and bones and one important diagnostic clue is the coexistence of lytic bone lesions and chylous effusion. An isolated presentation usually carries a better prognosis than does multi-organ involvement; the combination of pleural and peritoneal involvement with chylous effusions and lytic bone lesions carries the least favorable prognosis.

When lung involvement is suspected, high resolution computed tomography (HRCT) scans may reveal a diffuse liquid-like infiltration in the mediastinal and hilar soft tissue, resulting from diffuse proliferation of lymphatic channels and accumulation of lymphatic fluid; diffuse peribronchovascular and interlobular septal thickening; ground-glass opacities; and pleural effusion. Pulmonary function testing reveals either restrictive pattern or a mixed obstructive/restrictive pattern. While x-rays, HRCT scan, MRI, ultrasound, lymphangiography, bone scan, and bronchoscopy all can have a role in identifying lymphangiomatosis, biopsy remains the definitive diagnostic tool.

Microscopic examination of biopsy specimens reveals an increase in both the size and number of thin walled lymphatic channels along with lymphatic spaces that are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis, and possibly underlying fascia and skeletal muscle. Additionally, Tazelaar, et al., described a pattern of histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL).

Recognition of the disease requires a high index of suspicion and an extensive workup. Because of its serious morbidity, lymphangiomatosis must always be considered in the differential diagnosis of lytic bone lesions accompanied by chylous effusions, in cases of primary chylopericardium, and as part of the differential diagnosis in pediatric patients presenting with signs of interstitial lung disease.

The cornerstone of prevention and treatment of podoconiosis is avoidance of exposure to irritant soils. Wearing shoes in the presence of irritant soils is the primary method of exposure reduction. In Rwanda, a country of high disease prevalence, the government has banned walking barefoot in public, in order to curtail podoconiosis and other soil-borne diseases.

Once the disease has developed, rigorous foot hygiene including daily washing with soap and water, application of an emollient, and nightly elevation of the affected extremity has been shown to reduce swelling and disability. Compression wrapping and decongestive physiotherapy of the affected extremity has been shown to be effective in other forms of lymphedema, but the benefits of these therapies have not been rigorously studied in podoconiosis. Nodules will not resolve with these conservative measures, although surgical removal of the nodules can be performed.

Many lymphoceles are asymptomatic. Larger lymphoceles may cause symptoms related to compression of adjacent structures leading to lower abdominal pain, abdominal fullness, constipation, urinary frequency, and edema of the genitals and/or legs. Serious sequelae could develop and include infection of the lymphocele, obstruction and infection of the urinary tract, intestinal obstruction, venous thrombosis, pulmonary embolism, chylous ascites and lymphatic fistula formation.

On clinical examination the skin may be reddened and swollen and a mass felt. Ultrasonography or CT scan will help to establish a diagnosis.

Other fluid collections to be considered in the differential diagnosis are urinoma, seroma, hematoma, as well as collections of pus. Also, when lower limb edema is present, venous thrombosis needs to be considered.

Cases of lymphangioma are diagnosed by histopathologic inspection. In prenatal cases, cystic lymphangioma is diagnosed using an ultrasound; when confirmed amniocentesis may be recommended to check for associated genetic disorders.

The diagnosis usually is made serologically (through complement fixation) and by exclusion of other causes of inguinal lymphadenopathy or genital ulcers. Serologic testing has a sensitivity of 80% after 2 weeks. Serologic testing may not be specific for serotype (has some cross reactivity with other chlamydia species) and can suggest LGV from other forms because of their difference in dilution, 1:64 more likely to be LGV and lower than 1:16 is likely to be other chlamydia forms (emedicine).

For identification of serotypes, culture is often used. Culture is difficult. Requiring a special medium, cycloheximide-treated McCoy or HeLa cells, and yields are still only 30-50%. DFA, or direct fluorescent antibody test, PCR of likely infected areas and pus, are also sometimes used. DFA test for the L-type serovar of C trachomatis is the most sensitive and specific test, but is not readily available.

If polymerase chain reaction (PCR) tests on infected material are positive, subsequent restriction endonuclease pattern analysis of the amplified outer membrane protein A gene can be done to determine the genotype.

Recently a fast realtime PCR (TaqMan analysis) has been developed to diagnose LGV. With this method an accurate diagnosis is feasible within a day. It has been noted that one type of testing may not be thorough enough.

Reinke's edema is often diagnosed by an Ear, Nose & Throat (ENT) specialist or an Otolaryngologist by examination of the vocal cords. First, the doctor will review the patient's medical history and symptoms, such as hoarseness, dysphonia, and reduced vocal range. There is no familial or hereditary link to Reinke's edema. Because Reinke's edema is linked heavily to smoking, the doctor will need to know if the patient is a habitual smoker. Once the patient's history is reviewed, the vocal cords will be visualized using laryngoscopy, a technique in which a tube with a camera (endoscope) is passed through the nose and down the larynx. Laryngoscopes can be rigid or flexible. Flexible laryngoscopes, such as fiber laryngoscopes, allow the patient to produce sound as the tube is placed, and therefore allows the doctor to visualize movement of the vocal cord.

Based on the results of the laryngoscopy, Reinke's edema can be classified using a standardized system set in place by Yonekawa. This system characterizes the disease based disease .

Yonekawa Classification:

- Grade I – Lesions contact the anterior third of the vocal fold

- Grade II – Lesions contact the anterior two-thirds of the vocal fold

- Grade III – Lesions contact the entirety of the vocal fold

If further evaluation is needed, stroboscopy is used to examine mucosal waves of the vocal cords. Mucosal waves describe the waves produced by vibration of the vocal cords during speech. Stroboscopes produce flashes of light that are timed to the patient's vocal frequency. Every time the light is flashed, it will create a still frame image of the vocal cords at that particular moment in time. These are combined to produce an image of the wave. In the case of Reinke's edema, structural changes to the vocal cords will result in abnormal wave patterns.

Lymphohemangioma, or lymphohaemangioma, () is a disease characterized by swelling of the lymph nodes and blood vessels. It is variously described as a "mixture of clear fluid and blood-filled cysts", a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels. It is also sometimes described as a misnomer for combined lymphatic and capillary malformation.

Lymphohemangioma commonly appears in and around the eyes and mouth, but may also occur in other areas, including the pelvis, urethra, bladder, and in the limbs. Amputation of extremities may be necessary if the disease renders the extremity non-functional.

Thoracocentesis, pericardiocentesis, pleurodesis, ligation of thoracic duct, pleuroperitoneal shunt, radiation therapy, pleurectomy, pericardial window, pericardiectomy, thalidomide, interferon alpha 2b, Total Parenteral Nutrition (TPN), medium chain triglyceride (MCT) and high protein diet, chemotherapy, sclerotherapy, transplant;

Chyloderma is swelling of the scrotum resulting from chronic lymphatic obstruction. Obstruction may be caused by a nematode such as "Wuchereria bancrofti". This condition is also known as lymphscrotum or elephantiasis scroti.

Ainhum is an acquired and progressive condition, and thus differs from congenital annular constrictions. Ainhum has been much confused with similar constrictions caused by other diseases such as leprosy, diabetic gangrene, syringomyelia, scleroderma or Vohwinkel syndrome. In this case, it is called pseudo-ainhum, treatable with minor surgery or intralesional corticosteroids, as with ainhum. It has even been seen in psoriasis or it is acquired by the wrapping toes, penis or nipple with hairs, threads or fibers. Oral retinoids, such as tretinoin, and antifibrotic agents like tranilast have been tested for pseudo-ainhum. Impending amputation in Vohwinkel syndrome can sometimes be aborted by therapy with oral etretinate. It is rarely seen in the United States but often discussed in the international medical literature.

Soft tissue constriction on the medial aspect of the fifth toe is the most frequently presented radiological sign in the early stages. Distal swelling of the toe is considered to be a feature of the disease. In grade III lesions osteolysis is seen in the region of the proximal interphalangeal joint with a characteristic tapering effect. Dispersal of the head of the proximal phalanx is frequently seen. Finally, after autoamputation, the base of the proximal phalanx remains. Radiological examination allows early diagnosis and staging of ainhum. Early diagnosis is crucial to prevent amputation.

Doppler shows decreased blood flow in posterior tibial artery.

The first step in treating Reinke’s edema is to eliminate or control those risk factors that are causing the disease. This includes the cessation of smoking, the control of gastric reflux using antacids and/or Proton Pump Inhibitors (PPIs), and the discontinuation of activities that cause vocal distress. Those experiencing a hoarseness of the voice may choose to undergo voice therapy to improve the voice’s quality and range. Most cases of Reinke’s edema are caused by the long term usage of cigarettes. In this case, it is important to make lifestyle changes to stop smoking. While this will not resolve or improve the edema, the cessation of smoking will halt the disease's progression.

If the elimination of risk factors is not sufficient to improve the patient’s symptoms, surgery may be required. The most common type of surgery performed today for Reinke's edema is called surgical microlaryngoscopy. Most procedures follow the microflap technique set in place by Hirano. During surgery, an incision is made into the vocal cord using either microscissors or a CO laser. A flap of mucosa is lifted and the affected tissue is removed using suction or a microdebrider. The flap is then re-draped and trimmed to the appropriate size.

Most cases of Reinke’s Edema are bilateral - effecting both vocal cords - rather than unilateral. In the case of bilateral edema, the surgeon must choose whether to operate each side of the vocal cord in two separate surgeries or to operate both sides in a single surgery. The complication associated with removing tissue from both sides in a single surgery is that the raw, cut ends of the vocal cords may form an anterior glottis web, in which the two sides grow together in a continuous sheet. Other complications of surgery include tissue scarring due to damage to the vocal ligament during the incision and vocal cord stiffening due to over-suctioning of the superficial lamina propria (Reinke’s space).

While surgical microlarynscopy has its associated risks, if left untreated, Reinke’s edema can lead to a variety of long-term complications. Besides dysphonia (impaired speech), the most serious of these complications is airway obstruction due to severe inflammation of the vocal cords. The risk of complications has decreased drastically with the creation of new tools, such as the CO laser for surgical microlaryngoscopy. Before the Hirano microflap method was developed in 1895, vocal stripping was the most common procedure used to correct Reinke's Edema. Vocal stripping was often performed without magnification and with a monocular laryngoscope, instead of a binocular scope. This led to major complications such as vocal ligament scarring.

Women are more likely than men to undergo surgery due to a greater change in vocal pitch and quality. Surgery is capable of restoring the voice, with the condition that smoking is not resumed after surgery. Post-operative voice therapy is also advised to restore the voice's strength. Reinke's edema is not a fatal pathology unless the tissue becomes precancerous.

Congenital anomaly causes abnormal proliferation and dilation of lymphatic channels.

Affected persons are usually young adults that present with progressive dyspnea.

On radiological studies, diffuse lesions are visualized throughout the thoracic cavity using CT scans. The location of the lesions is mostly in the upper lobes of the lungs, usually in a lymphatic distribution. Thickening of the pleura and interlobular septal is also evident. In addition, pleural/pericardial effusions and mediastinal fat infiltration is appreciated. Definitive diagnosis is achieved through tissue biopsy.

Thoracocentesis and pericardiocentesis are performed to remove excess fluid. There is evidence in the literature that chemotherapy and radiation therapy helps to improve symptoms.

The prognosis for lymphangioma circumscriptum and cavernous lymphangioma is generally excellent. This condition is associated with minor bleeding, recurrent cellulitis, and lymph fluid leakage. Two cases of lymphangiosarcoma arising from lymphangioma circumscriptum have been reported; however, in both of the patients, the preexisting lesion was exposed to extensive radiation therapy.

In cystic hygroma, large cysts can cause dysphagia, respiratory problems, and serious infection if they involve the neck. Patients with cystic hygroma should receive cytogenetic analysis to determine if they have chromosomal abnormalities, and parents should receive genetic counseling because this condition can recur in subsequent pregnancies.

Complications after surgical removal of cystic hygroma include damage to the structures in the neck, infection, and return of the cystic hygroma.

Lymphatic malformations may be detected in the human fetus by ultrasound if they are of sufficient size. Detection of a cystic malformation may prompt further investigation, such as amniocentesis, in order to evaluate for genetic abnormalities in the fetus. Lymphatic malformations may be discovered postnatally or in older children/adults, and most commonly present as a mass or as an incidental finding during medical imaging.

Verification of the diagnosis may require more testing, as there are multiple cystic masses that arise in children. Imaging, such as ultrasound or MRI, may provide more information as to the size and extent of the lesion.