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Source: BSN medical research. There are two types of lymphedema - primary lymphedema and secondary lymphedema. Both types relate to lymph vessels or lymph nodes that are missing, damaged, removed or otherwise impaired.

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Primary lymphedema occurs when the lymph system is damaged due to a developmental defect. Primary lymphedema can be classified according to age of onset. Secondary lymphedema occurs due to an outside factor damaging or impairing the lymph system. Causes of secondary lymphedema include:. CDT is divided into two phases:. Visit our online catalog to browse the full line of JOBST compression products for management of edema, venous and lymphatic disease. All rights reserved.

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What is Lymphedema Learn more about the different types of lymphedema and their effects on the body. Lymphedema Types There are two types of lymphedema - primary lymphedema and secondary lymphedema. Primary Lymphedema Primary lymphedema occurs when the lymph system is damaged due to a developmental defect.

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Secondary Lymphedema Secondary lymphedema occurs due to an outside factor damaging or impairing the lymph system. Radiation treatment can damage the fragile lymphatic vessels and cause lymphedema. Tumors: Tumors can grow and press on the lymph vessels, blocking flow and causing lymphedema. Tumors often cause lymphedema which differs in that it starts in the upper part of the limb. Approximately cases have been reported in the medical literature. Symptoms of the following disorders can be similar to those of hereditary lymphedema. Comparisons may be useful for a differential diagnosis.

Secondary lymphedema is an acquired disorder that results from obstruction or damage to the lymphatic system. Affected individuals experience swelling due to the accumulation of lymph in the affected area. Secondary lymphedema may occur because of trauma, tumors, or after radiation therapy for cancer or surgery to remove lymph nodes. The most common cause of secondary lymphedema in tropical and subtropical regions is filarial infection in which lymphatic obstruction is caused by a parasitic disease known as lymphatic filariasis.

Lymphatic filariasis is caused by three different species of worms known as Brugia malayi, Brugia timori and Wuchereria bancrofti. These worms cause damage and inflammation to the lymphatic system. The worms are introduced into the human body through the bite of infected mosquitoes.

Secondary lymphedema is treated by addressing the underlying condition. For example, in lymphatic filariasis, certain drugs are used to kill filarial larvae and worms. For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database. Researchers have determined that hereditary lymphedema type II Meige syndrome and three other disorders occur due to different mutations of the same gene FOXC2.

These disorders include yellow nail syndrome, distichiasis-lymphedema syndrome, and lymphedema-ptosis syndrome.

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The exact relationship between these disorders is unknown. Yellow nail syndrome is a rare disorder characterized by yellow, thickened, and curved nails with almost complete stoppage of nail growth. Loss of the strip of hardened skin at the base and sides of a fingernail cuticles may also occur. Separation of the nails from the nail bed onycholysis may cause the nails to fall out. Yellow nail syndrome is usually associated with the presence of fluid in the lungs plural effusion and swelling of the arms and legs lymphedema.


Lymphedema usually occurs around puberty. Yellow nail syndrome occurs because of mutations to the FOXC2 gene and is inherited as an autosomal dominant trait.

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  • Distichiasis-lymphedema syndrome is a rare autosomal dominant multisystem disorder characterized by swelling due to fluid accumulation that usually occurs around puberty and the development of extra eyelashes along the posterior border of the lid margins distichiasis. Distichiasis may range from a few extra lashes to a full set of extra eyelashes.

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    • Swelling most often affects the legs. Additional anomalies associated with this disorder include cleft palate, droopy eyelids ptosis , abnormalities of the curved transparent outer layer of fibrous tissue covering the eyeball cornea , cysts on the spinal cord, an abnormal sensitivity to light photophobia , and cardiac heart defects. Distichiasis-lymphedema syndrome is caused by mutations of the FOXC2 gene and is inherited as an autosomal dominant trait.

      Lymphedema-ptosis syndrome is an extremely rare genetic disorder characterized by swelling because of fluid accumulation and droopy eyelids ptosis. Lymphedema usually occurs at or shortly after puberty. Lymphedema-ptosis syndrome occurs because of mutations of the FOXC2 gene and is inherited as an autosomal dominant trait.

      The diagnosis of hereditary lymphedema may be confirmed by a thorough clinical evaluation and a variety of specialized imaging tests including lymphoscintigraphy, ultrasound, and magnetic resonance imaging MRI. During lymphoscintigraphy, a radioactively labeled colloid substance is injected intradermally into either the hands or feet. The time required for the tracer to be transported from the point of injection to the regional lymph nodes is recorded.

      During an ultrasound, reflected sound waves create an image of the developing fetus. An ultrasound is used to rule out other conditions. A Doppler ultrasound can evaluate venous conditions such as varicose veins and venous blood clots. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues.

      An MRI is used to detect findings characteristic of hereditary lymphedema including swelling edema , a mass surrounded by a sac containing lymph fluid lymphocele , and the formation of fibrous tissue fibrosis. No gene therapy for hereditary lymphedema is currently available. There is no FDA approved medication to treat lymphedema. Lymphedema risk reduction practices should be followed to reduce complications such as infection and an increase in swelling.

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      Treatment is aimed at reducing swelling and preventing infection. Complete decongestive therapy CDT is a form of treatment in which specialized manual techniques manual lymph drainage is combined with multilayered compression bandaging, meticulous skin care, exercise, and the use of well-fitted compression garments.. Decongestive and conditioning exercises are important components of CDT. Antibiotics can be used to treat infections such as cellulitis or as a preventive prophylactic measure in individuals with recurrent infections.

      Various surgical techniques have been used to treat individuals with hereditary lymphedema including the surgical joining of small lymphatic vessels to nearby small veins microsurgical anastomosis has had some limited success in people with lymphedema. According to the medical literature, these therapies have had only limited effectiveness. Reducing operations are available to remove excess fibrotic tissue in cases of severe lymphedema. Continued use of compression garments is necessary after reducing surgery. Liposuction has not been found to be effective in primary lymphedema.

      Individuals with hereditary lymphedema should avoid long periods of immobility with legs in a dependent position. Affected individuals should also take special care to avoid wounds in any affected area because of a reduced resistance to infection. Excessive salt intake can cause fluid retention. Genetic counseling will benefit people with hereditary lymphedema and their families. Rehabilitation therapy may be necessary in cases where extreme lymphedema impairs daily activities. Information on current clinical trials is posted on the Internet at www.

      All studies receiving U. For information about clinical trials sponsored by private sources, contact: www. Botanicals such as the Benzopyrones and Saponins e. Benzopyrones, a group of substances such as coumarin, hydroxethylrutin and flavinoid derivatives, have been used for the treatment of individuals with hereditary lymphedema.

      These drugs breakdown proteins found in lymph and may stimulation lymph flow thereby reducing lymph accumulation and subsequent swelling. However, the effectiveness of such medications is unproven and under debate. More research is necessary to determine the long-term effectiveness and safety of benzopyrone therapy in individuals with hereditary lymphedema. Occasionally, drugs that promote fluid mobilization i. These medications increase urinary output and may help to reduce swelling in some affected individuals. However, diuretics have not been proven successful in reducing the swelling in primary lymphedema but may be beneficial in patients with mixed origin edema, e.

      The prolonged use of diuretics for the treatment of hereditary lymphedema should be carefully directed by a physician as these medications may have several long-term side effects. Joseph L. Weissleder H, Schuchhardt C, eds. Lymphedema Diagnosis and Therapy. Essen, Germany: Viavital Verlag; Chapters 5 and 8.